C ag e3 81 . Di om Fo i r d E git ng a et d l So ai it -O o ls, io n se n nl : ep s y cancercontroljournal.org Vol. 22, No. 4, October 2015 H. LEE MOFFITT CANCER CENTER & RESEARCH INSTITUTE, AN NCI COMPREHENSIVE CANCER CENTER Integrating Palliative Care Into Oncology: A Way Forward Improving the Quality of Palliative Care Through National and Regional Collaboration Efforts Palliative Pharmacotherapy: State-of-the-Art Management of Symptoms in Patients With Cancer Pharmacological Management of Cancer-Related Pain Clinical Implications of Opioid Pharmacogenomics in Patients With Cancer Palliative Sedation in Patients With Cancer Integrating Psychosocial Care Into Routine Cancer Care Systematic Review of Palliative Care in the Rural Setting New Frontiers in Outpatient Palliative Care for Patients With Cancer Palliative Care in Adolescents and Young Adults With Cancer Palliative Care in Older Patients With Cancer Prognostication of Survival in Patients With Advanced Cancer: Predicting the Unpredictable? Cancer Control is included in Index Medicus/MEDLINE Jacksonville, FL Permit No. 4390 H LEE MOFFITT CANCER CENTER & RESEARCH INSTITUTE INC 12902 Magnolia Drive Tampa FL 33612-9416 PAID NON-PROFIT ORG U.S. POSTAGE save the date SAVE THE D a n4n u a A l NNUAL Moffitt Cancer Center 4 th TH S TAT E - O F -T H E - A R T Neuro-Oncology c o n f eCONFERENCE rence March 10-11 Sheraton Sand K Clearwater Beac CONFERENC HIGHLIGHTS • Renownedfaculty representingthe leadinginstitution •Updatesonbrain spinetumors •Preconferencedin presentationonM •Interactivecasep withdiscussion • Physician,nursing pharmacycredito COURSE DIRECTORS Peter Forsyth, MD • Frank Vrionis, MD, MPH, PhD CONFERENCE CONTACT Marsha.Moyer@Moffitt.org • MOFFITT.org/NeuroOncology20 PROVIDED BY Editorial Board Members Editor: Lodovico Balducci, MD Senior Member Program Leader, Senior Adult Oncology Program Moffitt Cancer Center Deputy Editor: Julio M. Pow-Sang, MD Senior Member Chair, Department of Genitourinary Oncology Director of Moffitt Robotics Program Moffitt Cancer Center Editor Emeritus: John Horton, MB, ChB Professor Emeritus of Medicine & Oncology Guest Editors: Diane Portman, MD Assistant Member Department Chair, Supportive Care Medicine Program Leader, Palliative Medicine Moffitt Cancer Center Sarah Thirlwell, MSc, MSc(A), RN Nurse Director, Supportive Care Medicine Department of Supportive Care Medicine Moffitt Cancer Center John V. Kiluk, MD Associate Member Breast Oncology Richard D. Kim, MD Associate Member Gastrointestinal Oncology Bela Kis, MD, PhD Assistant Member Diagnostic Radiology Rami Komrokji, MD Associate Member Malignant Hematology Conor C. Lynch, PhD Assistant Member Tumor Biology Kristen J. Otto, MD Assistant Member Head & Neck Oncology Production Team: Veronica Nemeth Editorial Coordinator Sherri Damlo Medical Copy Editor Diane McEnaney Graphic Design Consultant Associate Editor of Educational Projects John M. York, PharmD Akita Biomedical Consulting 1111 Bailey Drive Paso Robles, CA 93446 Phone: 805-238-2485 Fax: 949-203-6115 E-mail: johnyork@akitabiomedical.com Michael A. Poch, MD Assistant Member Genitourinary Oncology For Consumer and General Advertising Information: Jeffery S. Russell, MD, PhD Assistant Member Endocrine Tumor Oncology Veronica Nemeth Editorial Coordinator Cancer Control: Journal of the Moffitt Cancer Center 12902 Magnolia Drive – MBC-JRNL Tampa, FL 33612 Phone: 813-745-1348 Fax: 813-449-8680 E-mail: Veronica.Nemeth@Moffitt.org Elizabeth M. Sagatys, MD Assistant Member Pathology - Clinical Moffitt Cancer Center Journal Advisory Committee: Saïd M. Sebti, PhD Senior Member Drug Discovery Daniel A. Anaya, MD Associate Member Gastrointestinal Oncology Bijal D. Shah, MD Assistant Member Malignant Hematology Aliyah Baluch, MD Assistant Member Infectious Diseases Lubomir Sokol, MD, PhD Senior Member Hematology/Oncology Dung-Tsa Chen, PhD Associate Member Biostatistics Hatem H. Soliman, MD Assistant Member Breast Oncology Hey Sook Chon, MD Assistant Member Gynecological Oncology Jonathan R. Strosberg, MD Assistant Member Gastrointestinal Oncology Jasreman Dhillon, MD Assistant Member Pathology - Anatomic Sarah W. Thirlwell, MSc, MSc(A), RN Nurse Director Supportive Care Medicine Program Jennifer S. Drukteinis, MD Associate Member Diagnostic Radiology Eric M. Toloza, MD, PhD Assistant Member Thoracic Oncology Clement K. Gwede, PhD Associate Member Health Outcomes & Behavior Nam D. Tran, MD Assistant Member Neuro-Oncology Sarah E. Hoffe, MD Associate Member Radiation Oncology Jonathan S. Zager, MD Associate Member Sarcoma and Cutaneous Oncology Cancer Control is a member of the Medscape Publishers’ Circle®, an alliance of leading medical publishers whose content is featured on Medscape (www.medscape.com). Most issues and supplements of Cancer Control are available at cancercontroljournal.org Cancer Control: Journal of the Moffitt Cancer Center (ISSN 1073-2748) is published by H. Lee Moffitt Cancer Center & Research Institute, 12902 Magnolia Drive, Tampa, FL 33612. Telephone: 813-745-1348. Fax: 813-449-8680. E-mail: ccjournal@Moffitt.org. Internet address: cancercontroljournal.org. Cancer Control is included in Index Medicus ®/MEDLINE® and EMBASE®/ Excerpta Medica, Thomson Reuters Science Citation Index Expanded (SciSearch®) and Journal Citation Reports/Science Edition. Copyright 2015 by H. Lee Moffitt Cancer Center & Research Institute. All rights reserved. Cancer Control: Journal of the Moffitt Cancer Center is a peer-reviewed journal that is published to enhance the knowledge needed by professionals in oncology to help them minimize the impact of human malignancy. Each issue emphasizes a specific theme relating to the detection or management of cancer. The objectives of Cancer Control are to define the current state of cancer care, to integrate recently generated information with historical practice patterns, and to enlighten readers through critical reviews, commentaries, and analyses of recent research studies. Disclaimer: All articles published in this journal, including editorials and letters, represent the opinions of the author(s) and do not necessarily reflect the opinions of the editorial board, the H. Lee Moffitt Cancer Center & Research Institute, Inc, or the institutions with which the authors are affiliated unless clearly specified. The reader is advised to independently verify the effectiveness of all methods of treatment and the accuracy of all drug names, dosages, and schedules. Dosages and methods of administration of pharmaceutical products may not be those listed in the package insert and solely reflect the experience of the author(s) and/or clinical investigator(s). October 2015, Vol. 22, No. 4 Cancer Control 377 Table of Contents Letter From the Editors A New Step Rather Than a New Era 381 Lodovico Balducci, MD, and Julio M. Pow-Sang, MD Editorial Perspectives, Progress, and Opportunities for Palliative Care in Oncology 382 Diane Portman, MD, and Sarah Thirlwell, MSc, MSc(A), RN Articles Integrating Palliative Care Into Oncology: A Way Forward 386 Kavitha Ramchandran, MD, Erika Tribett, MPH, Brian Dietrich, MD, and Jamie Von Roenn, MD Improving the Quality of Palliative Care Through National and Regional Collaboration Efforts 396 Arif H. Kamal, MD, MHS, Krista L. Harrison, PhD, Marie Bakitas, DNSc, CRNP, J. Nicholas Dionne-Odom, PhD, Lisa Zubkoff, PhD, Imatullah Akyar, PhD, RN, Steven Z. Pantilat, MD, David L O’Riordan, PhD, Ashley R. Bragg, Kara E. Bischoff, MD, and Janet Bull, MD Palliative Pharmacotherapy: State-of-the-Art Management of Symptoms in Patients With Cancer 403 Eric E. Prommer, MD Pharmacological Management of Cancer-Related Pain 412 Eric E. Prommer, MD Clinical Implications of Opioid Pharmacogenomics in Patients With Cancer 426 Gillian C. Bell, PharmD, Kristine A. Donovan, PhD, and Howard L. McLeod, PharmD Palliative Sedation in Patients With Cancer 433 Marco Maltoni, MD, and Elisabetta Setola, MD 378 Cancer Control October 2015, Vol. 22, No. 4 Table of Contents Articles, continued Integrating Psychosocial Care Into Routine Cancer Care 442 Paul B. Jacobsen, PhD, and Morgan Lee, MA Systematic Review of Palliative Care in the Rural Setting 450 Marie A. Bakitas, DNSc, CRNP, Ronit Elk, PhD, Meka Astin, MPH, Lyn Ceronsky, DNP, GNP, Kathleen N. Clifford, MSN, FNP-BC, J. Nicholas Dionne-Odom, PhD, RN, Linda L. Emanuel, PhD, MD, Regina M. Fink, RN, PhD, Elizabeth Kvale, MD, Sue Levkoff, ScD, MSW, Christine Ritchie, MD, MSPH, and Thomas Smith, MD New Frontiers in Outpatient Palliative Care for Patients With Cancer 465 Michael W. Rabow, MD, Constance Dahlin, ANP-BC, ACHPN, Brook Calton, MD, Kara Bischoff, MD, and Christine Ritchie, MD, MSPH Palliative Care in Adolescents and Young Adults With Cancer 475 Kristine A. Donovan, PhD, Dianne Knight, MD, and Gwendolyn P. Quinn, PhD Palliative Care in Older Patients With Cancer 480 Lodovico Balducci, MD, Dawn Dolan, PharmD, and Sarah A. Hoffe, MD Prognostication of Survival in Patients With Advanced Cancer: Predicting the Unpredictable? 489 David Hui, MD, MSc Departments Pathology Perspective: Is DOG1 Immunoreactivity Specific to Gastrointestinal Stromal Tumor? 498 William Swalchick, MA, Rania Shamekh, MD, and Marilyn M. Bui, MD, PhD Case Report: Occurrence of Multiple Tumors in a Patient 505 Special Report: Association of Cancer Stem Cell Markers With Aggressive Tumor Features in Papillary Thyroid Carcinoma 508 Elaine Tan, Mark Friedman, MD, and Domenico Coppola, MD Zhenzhen Lin, MD, Xuemian Lu, MD, Weihua Li, MD, Mengli Sun, MD, Mengmeng Peng, MD, Hong Yang, MD, Liangmiao Chen, MD, Chi Zhang, PhD, Lu Cai, MD, PhD, and Yan Li, MD, PhD October 2015, Vol. 22, No. 4 Cancer Control 379 Table of Contents Departments, continued Special Report: Should Vital Signs Be Routinely Obtained Prior to Intravenous Chemotherapy? Results From a 2-Center Study 515 Smitha Menon, MD, Nathan R. Foster, MS, Sherry Looker, RN, Kristine Sorgatz, RN, Pashtoon Murtaza Kasi, MBBS, Robert R. McWilliams, MD, and Aminah Jatoi, MD Special Report: Advancing Cancer Control Through Research and Cancer Registry Collaborations in the Caribbean 520 Rishika Banydeen, MPH, Angela M.C. Rose, MSc, Damali Martin, PhD, MPH, William Aiken, MD, Cheryl Alexis, MBBS, MSc, MRCP, Glennis Andall-Brereton, PhD, Kimlin Ashing, PhD, J. Gordon Avery, MB, ChB, MD, Penny Avery, SRN, Jacqueline Deloumeaux, MD, PhD, Natasha Ekomaye, Owen Gabriel, MD, Trevor Hassel, MBBS, Lowell Hughes, MBBS, Maisha Hutton, MSc, Shravana Kumar Jyoti, MD, Penelope Layne, RN/RM, Danièle Luce, PhD, Alan Patrick, MD, Patsy Prussia, MBBS, Juliette Smith-Ravin, PhD, Jacqueline Veronique-Baudin, PhD, Elizabeth Blackman, MPH, Veronica Roach, SRN, Camille Ragin, PhD Ten Best Readings Relating to Palliative Care for Oncology 531 Peer Reviewers, 2015 532 Index for 2015, Volume 22 533 About the art in this issue: Lisa Scholder is a multimedia artist whose canvas is the human body. Her technique involves applying body paint to nude models, then digitally photographing the painted body. The explosion of illuminating color on the human form is Scholder’s artistic trademark. All of the models shown in this issue are survivors of breast cancer of varying ages and body types, and they are part of the Bodies of Courage project. Scholder takes several hours to hand-paint the model with a crème-based paint and often incorporates other body-painted images in the final piece, which does not include the face of her model. Her artwork focuses on the abstract portrayal of the body infused with vibrant colors. Scholder’s late father-in-law, renowned Indian artist Fritz Scholder, had an unmistakable influence on her bold color combinations. Each model’s unique strength is represented with abstract and, at times, expressionist art forms on her body. The artist’s driving force is the self-empowerment that this process can bestow on her model, enabling her to see her body as a colorful, unique piece of art. With no formal art training, she began body painting in 2000 and developed her distinct body painting and photography style. Her first public exhibition was in 2004, and she progressed to gallery and art museum exhibitions since then. Bodies of Courage is an Arts in Medicine project (www.bodiesofcourage.org) in partnership with the Faces of Courage Foundation (www.facesofcourage.org), which provides day outings and overnight camps at no cost for women, children, and families diagnosed with any type of cancer. Lisa Scholder and Peggie D. Sherry, the founder of Faces of Courage, began this project 5 years ago as a way to raise awareness and as an artistic therapy for survivors of cancer. This project is an artistic testimony to the strength and determination of these survivors throughout their battles with cancer, their celebration of life, and their reconnection with the beauty of their own bodies. For information about the traveling gallery, please contact Peggie D. Sherry at psherry@facesofcourage.org or 813-948-7478. Further information on these projects is available at www.bodiesofcourage.org and www.facesofcourage.org. Cover: Linear. 20" × 24" Articles: Enlightened. 16" × 24" Road Between Art. 16" × 24" Sunlit Legs. 16" × 22" Solid Stance. 16" × 24" Greener Pastures. 16" × 22" Organic Walk. 16" × 24" Seizing the Light. 16" × 20" Masculine. 14" × 22" Family. 16" × 24" Sunlit. 16" × 24" Duality. 18" × 24" Dive Forward. 16" × 24" 380 Cancer Control October 2015, Vol. 22, No. 4 Letter From the Editors: A New Step Rather Than a New Era Dear Readers, Beginning with the January 2016 issue, Cancer Control: Journal of the Moffitt Cancer Center will be published in digital format only. With the launch of our new and improved website, readers will still be able to download and print the articles they wish to keep by visiting us at cancercontroljournal.org (formerly MOFFITT.org/ccj). The new URL links to our existing webpage under MOFFITT.org and will redirect readers to the new website as soon as it is available. Reprint requests will continue to be handled by the editorial office and will be reviewed and responded to on an individual basis. The decision to abandon the paper edition was based on considerations of cost effectiveness paramount for all fields of health care today. As the price of paper continues to rise and more and more people utilize electronic devices to read books, newspapers, and magazines, the increasing costs of printing no longer appear justified. Limited resources may be better utilized in maintaining and improving the quality and timeliness of our publication. For those of us who grew up in the pre-electronic era, who were used to waiting with anticipation for the new issue of our favorite medical journal in the mail, this change is a little disconcerting, as is the experience of seeing a daughter, now an adult, leave home and start her new family. It is disconcerting but necessary: as much as we regretted retiring our cherished stethoscope, we conceded that an echocardiogram was a more precise way to detect valvular disease or myocardial dysfunction than the human ear. Indeed, we plan to utilize the resources of the journal to fulfill our ongoing commitment to quality and practicality. Since its inception more than 20 years ago, Cancer Control promised to provide practicing oncologists with exhaustive and user-friendly reviews of important issues that could not be otherwise found in the literature. John Horton, MB, ChB, the founding editor and the quintessential clinical teacher, received universal praise for the impact Cancer Control had on the practice of oncology in the country and around the world. John C. Ruckdeschel, MD, the first chief executive officer of the H. Lee Moffitt Cancer Center & Research Institute and cofounder of Cancer Control, gave unlimited support to the educational mission of Moffitt and considered the journal to be the most effective means to fulfill this mission. The scope of oncology is rapidly enlarging and diversifying, and the practitioner is exposed to a barrage of new information that is both incomplete and contradictory. The main challenge of oncology education is to harness the energy of this scientific upsurge into a coherent discourse that highlights scientific advances together with new clinical questions. Fully committed to guide the practitioner to a safe exit from this informational maze, Cancer Control will devote the next issues to novel and timely topics. These include minimally invasive surgery, prospective radiosurgery and interventional radiology, imaging techniques based on biological markers, the use of genomic testing, the interpretation of results from clinical trials of targeted therapy, the use of mathematical models to predict tumor progression, overviews of tumor immunology and signal transduction, and the new scope of palliative care. Since establishing Cancer Control, Moffitt has grown to be the third largest comprehensive cancer center in the country. Basic, translational, and clinical investigators working at Moffitt illustrate the array of treatments and clinical trials available at our institution for the patients of Florida, the United States, and the world. A digital publication will allow us to focus unimpeded on the exciting and continual progress of cancer care. We would like to think that the adoption of a digital format represents a new step, rather than a new era, in the life of our journal — a step congruent with technological and scientific changes that will permit us to fulfill our mission in a rapidly changing world. Lodovico Balducci, MD Senior Member Program Leader, Senior Adult Oncology Program Editor, Cancer Control H. Lee Moffitt Cancer Center & Research Institute Tampa, Florida Lodovico.Balducci@Moffitt.org October 2015, Vol. 22, No. 4 Julio M. Pow-Sang, MD Senior Member and Chair, Department of Genitourinary Oncology Chief, Surgery Service Director, Moffitt Robotics Program Deputy Editor, Cancer Control H. Lee Moffitt Cancer Center & Research Institute Professor, Departments of Urology and Oncologic Sciences University of South Florida Morsani College of Medicine Tampa, Florida Julio.Powsang@Moffitt.org Cancer Control 381 Editorial Perspectives, Progress, and Opportunities for Palliative Care in Oncology In 2010, Temel et al1 demonstrated improved outcomes, including longer survival, in patients with metastatic non–small-cell lung cancer receiving palliative care along with usual oncology treatment. A provisional opinion published in 2012 by the American Society of Clinical Oncology further supported early palliative care for any cancer patient with advanced disease or high symptom burden.2 The Center to Advance Palliative Care, the American Cancer Society, and other national organizations have been instrumental in the advancement of the education, research, and literature base for supportive and palliative care in cancer settings. They have led the charge to support patient and family quality of life and align care with patient goals. Study results have demonstrated improved costs of care while maintaining quality, leading to significant advances in the penetration and growth of palliative care programs nationwide.3-6 However, more than a decade after the Institute of Medicine first studied the quality of cancer care, the obstacles to provision of quality palliative care for patients with cancer remain formidable. Patients frequently do not receive adequate symptom control or management of treatment-related side effects, and decisions about care often are not patient-centered or rooted in the most recent scientific evidence.7 In Dying in America, the Institute of Medicine’s 2014 consensus report on care of the dying, a committee of experts found that improving the quality and availability of medical and social services for patients and their families could not only enhance quality of life through the end of life, but may also contribute to a more sustainable care system.8 In this issue of Cancer Control, we present topics on palliative care that highlight the progress made to support the well-being of patients with cancer and the challenges to continued integration and advancement of the field of palliative care in oncology. Dr Ramchandran and colleagues review the compelling case for palliative care integration, the barriers to progress, and summarize key lessons gleaned from randomized controlled trials in palliative care integration in both the inpatient and outpatient oncology settings. Employing a case-study approach, they discuss means to integrate palliative care into oncology care and offer guidance for sustainability of integration. Among the strategies for scalable integration, the importance of quality measures and metric 382 Cancer Control development is emphasized. Dr Kamal and associates provide insight into quality improvement for palliative care achieved through collaborations that examine how care is delivered and may be improved. Their article describes endeavors to enhance the provision of quality palliative care at regional and national levels through cooperative efforts within an expansive group of community and academic palliative care providers. The development of the innovative, evidence-based Quality Data Collection Tool and its utilization are described as means to generate quality improvement projects aligned with national quality measurement initiatives. Such projects are an impetus for identifying and addressing troubling symptoms associated with serious illness, such as those frequently encountered in patients with cancer. The management of symptoms in cancer is updated in Dr Prommer’s article on state-of-the-art palliative pharmacotherapy. The treatment of prevalent symptoms that compromise the well-being of patients and their caregivers throughout the course of cancer care is emphasized, with particular focus on those that engender distress as disease progression occurs. Symptom mechanisms, means of assessment, and management approaches utilizing both medicationbased treatments and nonpharmacological therapies are described. In a second article, Dr Prommer reviews the pharmacological management of cancer pain. The World Health Organization analgesic ladder for the management of cancer pain of varying intensity is described, with detail provided on use of specific agents among the familiar “tried and true gold-standard” medications and more recently available agents. The additional value of adjuvants and interventional pain modalities is represented along with approaches to medication conversions and management of common opioid side effects. The epidemiology of malignant pain and the understanding of opioid responsiveness in the context of opioid receptor interactions are elucidated together with approaches to opioid-resistant pain. Given the use of opioids for cancer pain, Dr Bell and coauthors explore the basis of opioid analgesic responsiveness with a review of clinical studies that have assessed the connections between the effects of opioids and the genetic variants in the many genes October 2015, Vol. 22, No. 4 that govern their actions. The evidence is examined for associations between specific genetic variants and modulation of opioid response with variability in treatment results. Despite the challenges identified and the need for prospective studies comparing pharmacogenetic-guided opioid treatment to standard practice, the authors’ suggestion of the potential use of genotyping to achieve more effective therapy in cancer-related pain palliation is compelling. Dame Cicely Saunders, the founder of the modern hospice movement, emphasized the need for palliative care to manage “total pain”: The spiritual, psychological, social, and emotional elements that together with physical distress can cause intolerable suffering.9 Drs Maltoni and Setola review the controversial topic of palliative sedation for relief of refractory physical symptoms. Their article focuses on the application of proportionate palliative sedation at the end of life, consistent with national and international guidelines, as an ethical modality without effect on survival. The multifaceted understanding and management of total pain in cancer goes beyond relief of physical suffering and necessitates the integration of psychosocial care.10,11 Drs Jacobsen and Lee review progress in this area, describing the application of standards, key clinical practice guidelines, and quality monitoring. They describe the effect of such monitoring on quality in psychosocial care. Models are provided to demonstrate efforts to enhance provision of psychosocial care by implementing such standards and guidelines in community settings. Dr Bakitas and colleagues discuss the limitations to access and the dissemination of comprehensive palliative care for patients with cancer living in rural settings. They have gathered empirical evidence, largely focused on patients with cancer, and describe the present state in rural palliative care research and practice. The article reveals a dearth of research in this arena and a paucity of rural palliative care services, resulting in limited care. However, the successful initiatives described demonstrate opportunities to establish palliative care practice services and standards specific to rural settings. Dr Rabow and associates describe other areas of importance in community-based palliative care in their article about outpatient oncological palliative care. Recognizing the fundamental but minority role played by hospital palliative care in the context of the totality of palliative care required in oncology is pivotal. The article describes the current state in oncology palliative care and highlights vanguard elements in outpatient oncology palliative care, including the setting and timing of palliative care integration into outpatient oncology, quality and measurement, research, electronic and technical innovations, finances, October 2015, Vol. 22, No. 4 and the relationship between primary and specialty palliative care. Specialty palliative care distinguishes the activity of specialty-trained providers managing complex refractory symptoms, existential and psychosocial distress, medical futility, and advanced communications. However, specialty palliative care in oncology also encompasses expert palliative care that may be provided to special populations of patients with cancer who have unique needs. The particular challenges of these groups and their care are described in the articles on palliative care in adolescent and young adult patients with cancer by Dr Donovan and coauthors and palliative care of older patients with cancer by Dr Balducci and associates. The article by Donovan and colleagues highlights the limited provision of palliative care and research studies on palliative care in adolescent and young adult patients with cancer. Gaps in care with high potential for distress and opportunities for earlier inclusion of palliative care are also identified. The article features guidelines supporting the integration of palliative care, the options for advance care planning, and challenges to implementation in this patient population. Balducci et al focus on palliative care for older patients with cancer and provide a comprehensive overview of the effects of advancing age. They emphasize specialized palliative care concerns pertaining to this expanding population, a group also frequently affected by nononcological medical issues. The priorities elucidated include setting goals, prevention and management of treatment complications, management of cancer-related symptoms, and management of older survivors of cancer. Survival prediction principles and recent literature on prognostication are reviewed by Dr Hui in the context of examining clinician prediction of survival in patients with advanced cancer. With emphases on prognostication as a process, the evolution of prognostic factors over time, the variability in prognostic accuracy, and the overriding principle of unpredictability of the exact time of death, Dr Hui highlights the uncertainty in survival prediction. Yet, use of existing validated prognostic models and factors still enable clinicians to provide approximated time frames. These can facilitate clinical decision making in the present, and the future holds promise for multiple opportunities in prognostication research. We have compiled this compendium of topics in palliative care in oncology in the hopes of advancing our understanding and adoption of palliative care in cancer. Our goal is partnership to enhance patient and caregiver quality of life throughout the cancer continuum. We are gratified with the progress made and motivated by the opportunities that remain. Cancer Control 383 Diane Portman, MD Guest Editor Department Chair, Supportive Care Medicine Program Leader, Palliative Medicine H. Lee Moffitt Cancer Center & Research Institute Tampa, Florida Diane.Portman@Moffitt.org Sarah Thirlwell, MSc, MSc(A), RN Guest Editor Nurse Director, Supportive Care Medicine Department of Supportive Care Medicine H. Lee Moffitt Cancer Center & Research Institute Tampa, Florida Sarah.Thirlwell@Moffitt.org References 1. Temel JS, Greer JA, Muzikansky A, et al. Early palliative care for patients with metastatic non-small-cell lung cancer. N Engl J Med. 2010;363(8):733-742. 2. Smith TJ, Temin S, Alesi ER, et al. American Society of Clinical Oncology provisional clinical opinion: the integration of palliative care into standard oncology care. J Clin Oncol. 2012;30(8):880-887. 3. Morrison RS, Dietrich J, Ladwig S, et al. Palliative care consultation teams cut hospital costs for Medicaid beneficiaries. Health Aff (Millwood). 2011;30(3):454-463. 4. Parikh RB, Kirch RA, Smith TJ, et al. Early specialty palliative care — translating data in oncology into practice. N Engl J Med. 2013;369(24):2347-2351. 5. Penrod JD, Deb P, Dellenbaugh C, et al. Hospital-based palliative care consultation: effects on hospital cost. J Palliat Med. 2010;13(8):973-979. 6. Hughes MT, Smith TJ. The growth of palliative care in the United States. Annu Rev Public Health. 2014;35:459-475. 7. Levit L, Balogh E, Nass S, et al, ed. Delivering High-Quality Cancer Care: Charting a New Course for a System in Crisis. Washington, DC: National Academies Press; 2013. 8. Committee on Approaching Death. Dying in America: Improving Quality and Honoring Individual Preferences Near the End of Life. Washington, DC: National Academies Press; 2014. 9. Saunders C. Cicely Saunders: Selected Writings 1958-2004. New York: Oxford University Press; 2006. 10. Hallenbeck J, McDaniel S. Palliative care and pain management in the United States. In: Moore RJ, ed. Biobehavioral Approaches to Pain. New York: Springer; 2009:493-513. 11. Surbone A, Baider L, Weitzman TS, et al. Psychosocial care for patients and their families is integral to supportive care in cancer: MASCC position statement. Support Care Cancer. 2010;18(2):255-263. 384 Cancer Control October 2015, Vol. 22, No. 4 Department of Supportive Care Medicine H. Lee Moffitt Cancer Center & Research Institute The Department of Supportive Care Medicine at the H. Lee Moffitt Cancer Center & Research Institute is engaged in clinical care, training and research. The department includes the sections of Palliative, Behavioral, and Integrative Medicine. Consultative and management services are offered in both the outpatient and inpatient settings. The activities of the department address the physical, emotional, social, and spiritual challenges that occur throughout the course of cancer care. Department Chair Diane Portman, MD PALLIATIVE MEDICINE BEHAVIORAL MEDICINE INTEGRATIVE MEDICINE Diane Portman, MD Young D. Chang, MD Ritika Oberoi-Jassal, MD Sahana Rajasekhara, MD Joshua Smith, MD Cynthia Brown, ARNP Leah Crist, ARNP Laura Donovan, ARNP Ann Guastella, ARNP Jessica Latchman, ARNP David Craig, PharmD Robin Lawson, LCSW Mareda Kennedy, MDiv Margarita Bobonis, MD, Section Chief Barbara Lubrano di Ciccone, MD Marguerite Pinard, MD Margaret Booth-Jones, PhD Kristine Donovan, PhD Lora Thompson, PhD Lora Thompson, PhD, Director Liem Quang Le, LAc, Acupuncture Sharen Lock, Mindfulness & Yoga Mary Marton, Massage Therapy Elaine Payne, Massage Therapy Jonatha Wright, Massage Therapy Program Support Clinical Support Department Administrator Carol Knop, MBA Clinic Operations Manager Kathy Loufman, RN, MS Nurse Director Sarah Thirlwell, MSc, MSc(A), RN Registered Nurses Medical Assistants Patient Services Specialists Supervisor Peggy Kirwin Community and Patient Programs Education and Training Yoga Classes Meditation Classes Finding Balance with Cancer Program USF College of Medicine • Medical Students • Hospice and Palliative Medicine Fellows • Hematology-Oncology Fellows Breast Surgical Oncology Fellows International Visiting Scholars Management Assistants For more information about the Department of Supportive Care Medicine, call the department at 813-745-6045 (during normal business hours). www.MOFFITT.org October 2015, Vol. 22, No. 4 Cancer Control 385 Palliative care should be initiated early in the treatment continuum of cancer. Photo courtesy of Lisa Scholder. Enlightened. 16” × 24”. Integrating Palliative Care Into Oncology: A Way Forward Kavitha Ramchandran, MD, Erika Tribett, MPH, Brian Dietrich, MD, and Jamie Von Roenn, MD Background: Patients with cancer have complex physical, psychosocial, and spiritual needs that evolve throughout their disease trajectory. As patients are living longer with a diagnosis of cancer, the need is growing to address the morbidity due to the underlying illness as well as treatment-related adverse events. Palliative care includes treating physical symptoms as well as addressing psychosocial and spiritual needs. When these needs are addressed, the quality of care improves, costs decrease, and goals are aligned between the medical care provided and the patient and family. However, how best to integrate palliative care into oncology care is still an area of investigation. Methods: The authors conducted a literature search, including randomized clinical trials and practice reviews, to evaluate the evidence for integrating palliative care into oncology care. Barriers to integration as well as sustainable paths forward are highlighted. The authors also utilize case studies as representative examples of integration. Results: Current studies demonstrate that integrating palliative care into oncology care improves symptom control, rates of patient and family satisfaction, and quality of end-of-life care. However, for systemwide integration to be successful, commitment must be made to quality improvement, an infrastructure must be built to support palliative care screening, assessment, and intervention, and stakeholders must be engaged in the program. In addition, value must be demonstrated using metrics that affect quality, care utilization, and patient satisfaction. Conclusions: Even though most US cancer centers have a palliative care program, palliative care remains limited in scope. An integrated approach for palliative care with oncology care requires a systems-based approach, with agreement between all parties on shared common metrics for value. Introduction Significant progress has been made in the management and treatment of cancer, but morbidity rates continue From the American Society of Clinical Oncology (JV), Alexandria, Virginia, Stanford Cancer Institute (KR), and Stanford University School of Medicine (ET, BD), Palo Alto, California. Submitted April 15, 2015; accepted September 2, 2015. Address correspondence to Jamie Von Roenn, MD, American Society of Clinical Oncology, 2318 Mill Road, Suite 800, Alexandria, VA 22314. E-mail: Jamie.VonRoenn@asco.org No significant relationships exist between the authors and the companies/organizations whose products or services may be referenced in this article. 386 Cancer Control to increase.1,2 An estimated 1,658,370 new cancer diagnoses and 589,430 cancer-related deaths are expected to occur in the United States in 2015.3 These data reflect a 22% decline in overall cancer mortality, translating to approximately 1,519,300 fewer cancer-related deaths from 1991 to 2011.3,4 This decrease in mortality may be associated with an increase in cancer morbidity, because a growing number of survivors experience the late-onset and long-lasting effects of cancer and its treatment.2 Patients and families experience physical, psychological, and spiritual symptoms throughout the disease trajectory, sometimes leading to poor quality of life and suboptimal treatment outcomes.5 October 2015, Vol. 22, No. 4 Palliative care can help relieve symptoms and improve well-being in patients and their families living with a serious illness.6 The World Health Organization (WHO) defines palliative care as7: An approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual. Palliative care should be initiated at the point of diagnosis and continued throughout survivorship and end of life. Many pivotal studies have demonstrated improvements in outcomes when palliative care is integrated into the continuum of care, including quality of life, mood, the utilization of health care, patient satisfaction, patient understanding of prognosis, and survival.8-10 Call for the Integration of Palliative Care Major organizations, such as the American Society of Clinical Oncology (ASCO), National Comprehensive Cancer Network (NCCN), Institute of Medicine (IOM), WHO, and the European Society for Medical Oncology (ESMO), endorse the integration of palliative care into comprehensive cancer care.11-15 ASCO published a vision for optimal oncology care for 2020 that identified integrated palliative care as an essential component of optimal cancer care.16 In a provisional clinical opinion, ASCO recommended integrated care for all patients with metastatic disease or high symptom burden.11 NCCN developed guidelines to facilitate the integration of palliative and oncological care with schematics for screening, assessments, interventions, reassessment, and after-death care.12 IOM explains that involving palliative care is a requirement for comprehensive care.13 WHO developed guidelines in 1990 for providing palliative care to patients with malignancy.14 ESMO has been a strong proponent of integrating palliative and cancer care.15 As a component of this effort, it created an accreditation program to identify cancer programs with well-integrated palliative care services.15 Barriers Significant barriers still prevent the effective integration of palliative care into the continuum of cancer care. Such barriers include an inadequately trained workforce, cultural stigma (eg, palliative care equates to end-of-life care), lack of payment models supporting early and regular palliative care, and a need for well-defined metrics for quality palliative care.17 In addition, guidance on methods for integration is lacking. At a minimum, integrating palliative care into October 2015, Vol. 22, No. 4 cancer care requires 3 components: (1) routine identification of palliative care needs through screening, (2) standardized assessment of palliative care needs, and (3) management and treatment of identified needs. Clarity is lacking on how to accomplish all 3 areas.5,18,19 Moving from evidence to practice requires an understanding of the barriers and strategies to identify, assess, and manage palliative care needs. This review will focus on key lessons learned from randomized controlled trials focused on the integration of palliative care and provide possible strategies for implementing and integrating scalable, patient- and family-centered models of palliative care into cancer care. Evidence and Implications Providing concurrent palliative care and standard care for the management of cancer has proven benefits. Studies of integrated care in the inpatient/hospital or home setting have shown that the addition of palliative care to standard care reduces hospitalbased costs and improves the quality of end-of-life care and symptom management.17,20-22 Because medical oncology is increasingly provided in the ambulatory setting, more relevant data to assess the impact of integrating cancer and palliative care arise from studies performed in the outpatient setting. The results of 3 randomized controlled trials demonstrated improved outcomes for study participants randomized to the palliative care intervention.8-10 An investigation of these studies provides insights for the integration of palliative medicine into cancer care. Temel et al8 randomized 151 patients with newly diagnosed metastatic lung cancer to receive either early palliative care in conjunction with standard oncology care or standard oncology care alone. Early palliative care consisted of a visit with a palliative care team at intervals of at least 30 days, from the point of diagnosis until end of life.8 Outcomes measured included changes in mood and quality of life, aggressiveness of end-of-life care (chemotherapy ≤ 14 days before death, lack of hospice care, or admission to hospice ≤ 3 days before death), and understanding of prognosis.8 Study patients assigned to the integrated palliative care arm had significant improvement in quality-of-life scores compared with those assigned to the standard oncology care (P = .03), as well as fewer depressive symptoms (P = .01) and less-aggressive, end-of-life care (P = .05).8 Despite less-aggressive, end-of-life care, study patients in the concurrent palliative care arm had prolonged rates of survival compared with those assigned to the standard treatment arm (8.9 vs 11.6 months; P = .02).8 In addition, those in the integrated care arm had a more accurate understanding Cancer Control 387 of their prognosis, were more likely to have resuscitation preferences documented, and were less likely to receive chemotherapy at the end of life than their counterparts (9% vs 50%; P = .02).8 At baseline, approximately one-third of all study participants said they thought their disease could be cured with treatment.8 The investigators hypothesized that this decreased utilization of chemotherapy at the end of life may account for the improved survival rates. In Project Educate, Nurture, Advise, Before Life Ends, Bakitas et al9 led a randomized study of a telephone-based educational intervention in a rural setting. A total of 312 patients with advanced solid tumors and life-limiting prognoses (approximately 1 year) were randomized to receive usual care or usual care plus nursing intervention. The nursing intervention was based on a chronic care model that focused on patient empowerment and encouraged patient self-management and problem solving. It consisted of 4 weekly sessions focused on symptom management, communication and social support, problem solving, and advance care planning, followed by at least monthly telephone contact. Outcomes measured included symptom intensity, quality of life, mood, and resource use. Compared with those in the usual care group, those in the intervention group reported significant improvement in quality of life (P = .02), depression (P = .02), and a favorable trend in symptom intensity (P = .06). No difference was seen in the use of health care resources between the study groups, as measured by the number of emergency department visits or days in the hospital or intensive care unit. Bakitas et al concluded that a nurse-led intervention had the potential to impact quality of life and mood, but it was less likely to impact symptom intensity or resource utilization. Rather, they hypothesized that a more intensive intervention (eg, in person) might be more likely to significantly impact symptoms and resource use, although they admitted that such a model is less feasible for patients living in rural areas. A Canadian trial by Zimmerman et al10 also supports the finding that the early integration of palliative care into cancer care in the outpatient setting improves patient outcomes. This cluster-randomized controlled trial of 461 patients with advanced solid tumors from 24 clinics in Toronto, Canada, assessed the impact of early consultation and follow-up by a palliative care team in addition to standard oncology care compared with standard cancer care alone.10 Eligible participants had advanced cancer and a clinical prognosis of between 6 and 24 months.10 Those in the intervention arm had an initial consultation with a palliative care team and at least monthly followup visits.10 Outcomes measured included symptom severity, quality of life, satisfaction with care, and 388 Cancer Control patient/family difficulties communicating with their health care team.10 At 3 and 4 months, patients in the intervention arm reported improvements in quality of life (P = .05 and P = .003, respectively) and satisfaction with care (P = .0003 and P < .001, respectively).10 Improvement in symptom severity was noted at the 4-month time point alone (P = .05).10 The authors noted that quality of life is a broad construct that can improve, even when a change in symptoms is lacking.10 The randomized trials conducted by Temel et al,8 Bakitas et al,9 and Zimmermann et al10 provide key lessons for integrated practice, including (1) the benefits of normalizing the standard integration of palliative care for all patients, (2) the impact of integrating palliative care on the psychosocial domain of care, and (3) the need for sustainable management of palliative care resources and value-driven metrics. Normalizing Palliative Care Among the 3 randomized controlled trials presented, all patients in the intervention arms were connected to palliative or supportive consultation.8-10 Temel et al8 attributed some benefits of the intervention to immediate eligibility and enrollment of patients with a new diagnosis of advanced cancer. Also notable is that the study dropout rate was 1%, suggesting that, once initiated, utilization of palliative care may be driven by patient satisfaction, family member satisfaction, or both.8 A national poll conducted by the Center to Advance Palliative Care revealed that 7 out of 10 people are unfamiliar with palliative medicine, but, once it was described or offered to them, 92% said they would consider it as support to improve quality of life and symptom management.23 In addition, the results of a qualitative study from Schenker et al24 suggested that patients with unmet physical and psychosocial symptoms are likely to perceive a need for specialist palliative care. No significant association was seen between perceived need and likelihood to request palliative services.24 However, patients were more likely to see palliative specialists if their oncologist recommended the service.24 Based on these data, educating the public and establishing palliative care as a standard of care at the time of a cancer diagnosis is likely to be acceptable to patients and families. In addition, oncologists are key players in introducing patients to and supporting palliative care for those with an identified need.24 Psychosocial and Decision-Making Support Palliative medicine is traditionally associated with the management of physical symptoms and transitioning to end-of-life care.22 However, evidence suggests that palliative medicine can be a source of psychological and decision-making support.8-10 In the study by Temel et al,8 the palliative care clinicians in October 2015, Vol. 22, No. 4 the trial focused their attention on assessing psychosocial symptoms, establishing goals of care, assisting with decision-making regarding treatment, and coordinating care. This focus was associated with significant improvement in quality of life, decreased depressive symptoms, and greater understanding of prognosis among the study participants.8 Likewise, Zimmerman et al10 realized a more pronounced impact on quality of life than on physical symptoms as a result of the palliative care intervention. Bakitas et al9 also found a positive effect on quality of life and mood in the palliative care intervention group. A summary of evidence in support of the early integration of palliative care proposed the following dominant mechanisms by which palliative care may affect outcomes26: psychological support, knowledge of illness, and coping behaviors (Fig 1). Caring for psychosocial health, as well as empowering decision-making skills for patients and families, is integral to optimal cancer care. Sustainable Management of Palliative Resources Primary palliative care is defined as the “basic skills and competencies required of all physicians and other health care professionals.”27 For example, primary palliative care may include neuropathy management by an oncologist during routine care. Secondary palliative care refers to practices of “specialist physicians and organizations that provide consultation and specialty care.”27 Specialists may be consulted when Early, Integrated Palliative Care Model Practices and Processes • Team approach • Decision-making • Education/support Patient-Level Targets • Physical • Psychological • Sociocultural • Spiritual/existential • Ethical/legal Caregiver Support Oncology Team Palliative Care Team Specialist Palliative Care Primary Palliative Care Patient + Family Fig 2. — Patient-centered model of primary and specialist palliative care that leverages oncology and specialist teams. symptoms are challenging to control with initial interventions (eg, complex pain syndromes, refractory anxiety; Fig 2). Temel et al8 utilized a high-resource, specialist model in which patients met with their assigned palliative care clinicians in coordination with their oncologists. Alternatively, Bakitas et al9 described a Quality of Life Physical Symptoms Mood Illness, Knowledge, and Understanding Health Behaviors: Anticancer therapies End-of-life-care Survival Coping Behaviors Fig 1. — Hypothesized relationships between early palliative care interventions and clinical outcomes. From Irwin KE, Greer JA, Khatib J, et al. Early palliative care and metastatic non-small cell lung cancer: potential mechanisms of prolonged survival. Chron Respir Dis. 2013;10(1):35-47. Reprinted by permission of SAGE Publications. October 2015, Vol. 22, No. 4 Cancer Control 389 nurse-led approach that equipped the nursing team with palliative care skills, including didactic training and reference tools, and incorporated patient self-management. Both interventions realized significant improvements in quality of life and depression.8,9 Given the increased palliative care needs of people affected by cancer and the limited specialist workforce, models such as Project Educate, Nurture, Advise, Before Life Ends that utilize scripted interventions to build capacity for palliative care are critical to the sustainable provision of services.9 Scalable models for palliative care integration will require education of all health care professionals who provide primary palliative care as well as adequate support (eg, time, reimbursement). In addition, appropriate guidelines and referral criteria must be put into place for specialist support. Establishing Standard Measurements and Defining Value Integrating palliative care into oncology could have a higher likelihood of success if its value is clearly defined and accurately and consistently measured.28,29 In addition, clarity is needed on which components of a palliative care intervention affect which outcomes. For example, what interactions and with whom are absolutely necessary to achieve improved psychosocial well-being? The randomized studies by Temel et al,8 Bakitas et al,9 and Zimmermann et al10 all included well-defined primary and secondary outcome measures. Comparisons between interventions and future investigations require an accepted common set of quality indicators that reflect the goals and scope of the specialty. Several groups have proposed quality metrics for palliative care.28-31 ASCO’s Oncology Practice Initiative includes metrics on symptom measurement (pain, dyspnea, and constipation) as well as quality of end-of-life care (time on hospice, location of death).30 The Center to Advance Palliative Care also convened a panel to establish a common set of metrics to measure patient/family, clinical, operational, and financial outcomes (Table 1).31 Its consensus recommendations included metrics for symptom assessment, goals of care, family support, and transition management. It also recommended measurements of satisfaction among patients, their family members, and the health care team. Kamal et al28 reported on the landscape of quality measures relevant to palliative care utilizing domains defined by the National Quality Forum (Table 2). They identified metrics in physical, psychological, cultural, social, ethical, end of life, and spiritual care.28 Metrics in some studies have focused on the alleviation of physical symptoms, quality of life, and the quality of end-of-life care.8-10,21-23,32 However, these 390 Cancer Control measures are but a starting point; further delineation of cause and effect (eg, which component of the palliative care intervention affects which measure) is needed. Better understanding is needed of how patients, their families, and health care teams measure success in achieving optimal quality of living with a cancer diagnosis. Table 1. — Categories and Examples of Common Metrics to Assess Palliative Care Consultation Programs Categories Common Metrics Patient/family Satisfaction scores Clinical Symptom control scores Psychosocial assessment scores Operational Demographics Disease diagnosis and staging Referring health care professional Emergency department visits Hospital admissions/readmissions Hospital and intensive care unit lengths of stay Hospice referral Financial Daily pre- and post-consultation hospital cost Net loss/net gain for inpatient deaths From Weissman DE, Meier DE. Identifying patients in need of a palliative care assessment in the hospital setting: a consensus report from the Center to Advance Palliative Care. J Palliat Med. 2011;14(1):17-23. Copyright © 2011, reprinted by permission of Mary Ann Liebert, Inc. Table 2. — National Quality Forum Domains and Select Quality Metrics for Palliative Care in Oncology Care Domain Example Metrics Physical Symptom assessment and management (pain, dyspnea, fatigue, nausea) Psychological Psychosocial support Caregiver depression Depression Grief and bereavement Social General management Family satisfaction Family preferences Spiritual Spiritual support Value of life Spiritual need Cultural Communication needs Culturally sensitive care End of life Pain at end of life Information at end of life Death recognition Peace at death Ethical aspects Respect Insight into illness Impaired capacity Advance care planning Patient preferences From Kamal AH, Gradison M, Maguire JM, et al. Quality measures for palliative care in patients with cancer: a systematic review. J Oncol Pract. 2014;10(4):281-287. Copyright © 2014, reprinted by permission of American Society of Clinical Oncology. October 2015, Vol. 22, No. 4 Designing Sustainable Palliative Care: 2 Case Studies Several organizations have been using innovative approaches to develop models of integration that incorporate knowledge from randomized trials as well as an understanding of local systems, culture, and stakeholders.33-36 Cancer Care Ontario (CCO; Toronto, Ontario, Canada) and Stanford Health Care (Palo Alto, California) are 2 case studies that offer strategies for integrating palliative care into cancer care beginning with routine screening and assessment. Both include efforts to define the roles and resources required for primary and specialist palliative care to accomplish this and manage the needs of patients and their families. CCO implemented a provincewide screening for symptoms, whereas Stanford Health Care is establishing value-based palliative care at a single academic medical center.33,37 Both are using a participatory, quality-improvement approach. Cancer Care Ontario: Symptom Management CCO, which is the provincial agency responsible for continually improving cancer services, launched the Provincial Palliative Care Integration Project (PPCIP) to improve care through systemwide screening, assessment, and management of cancer-related symptoms.33 CCO developed this quality-improvement initiative based on evidence that better symptom management and collaborative care “improves the patient experience across the cancer journey.”38 In the first iteration of the PPCIP, the Edmonton Symptom Assessment Scale (ESAS) was used to screen for physical and psychosocial symptoms, and toolkits were developed to facilitate follow-up assessment and symptom management.39 The project was pilot tested in 1 clinic, but it expanded throughout the province to include more than 25,000 symptomintensity screenings.33 CCO established a consistent reporting mechanism, the Interactive System Assessment and Collection, which allowed regional cancer centers to track the success of ESAS as a tool and use patient data to inform treatment decisions.39 Interactive System Assessment and Collection is now embedded within electronic health records at 11 hospitals.39 It captures physical symptoms through ESAS and functional status using the Patient-Reported Functional Status tool.39 In 2008, the results were publically reported and symptom screening became a quarterly performance indicator for each regional cancer care program.33 Survey results published in 2012 reported that 89% of patients thought ESAS was important to complete, 79% thought their health care professionals used the results to help formulate their care plan, and 78% reported that their symptoms had been controlled to a comfortable level.33 As the CCO initiative evolved, health care professionals outside of the initial PPCIP October 2015, Vol. 22, No. 4 could view these results and request participation in the program.39 Participation increased from 6 hospitals in 2007 to 29 hospitals by 2013, and provincial screening has steadily increased, averaging 58% en route to their target of 70%.39 CCO provides a promising example of integrating at least 1 essential component of palliative care — symptom management — into oncology. It established a specific aim for symptom management, developed standard processes at the regional level, and created a transparent measurement system to track screening, symptom intensity, and functional status over time. In addition, it has normalized the integration of palliative care into routine cancer care from the point of diagnosis. Stanford Health Care: Value-Based Palliative Care In 2013, a patient with lymphoma receiving treatment at Stanford Health Care asked whether palliative care was supposed to be patient-centered. Indeed, the health care delivery model was not designed with a patient or family member in mind. The conversation prompted a design initiative at the Stanford Cancer Institute to improve quality of life for all 3 stakeholders — patients, their family members, and the health care team — by incorporating their values into the core processes and measures of palliative care delivery. This project is 1 component of the Stanford Cancer Institute Transformation Initiative, a joint project of the School of Medicine and Stanford Health Care to transform the experience of patients with cancer through comprehensive, coordinated, and compassionate care.37 Stanford Health Care aims to routinely identify, assess, and manage the palliative care needs of patients and families from the point of diagnosis through survivorship and end of life. The institutional target is 100% screening for palliative care needs utilizing a global screening tool, the Patient Reported Outcomes Measurement Information System, which is a patient reporting tool that evaluates physical, emotional, social, spiritual, and functional needs. The hypothesis is that the primary oncology team can manage 50% of palliative care needs, and 50% will be referred to specialist services. Data will be gathered via electronic medical records of Stanford Health Care and will be documented on a dashboard visible to all stakeholders. Stanford Health Care has engaged a diverse group (called the Core Innovation Team) of patients, family members, administrators, and physicians in oncology and palliative medicine to codesign strategies for primary and specialist palliative care. Baseline data to inform the work of the Core Innovation Team include interviews with patients and family members about their perceptions and knowledge of Cancer Control 391 palliative care as well as their personal values and goals. Physicians were interviewed to determine their understanding of palliative care, what components of palliative care they practiced, and when and how they integrated specialist palliative care. Physician values and goals were also assessed as well as how their own wellness might be improved in the context of their work. An initial design session with the Core Innovation Team and several external stakeholders, including experts from health care consulting, marketing, and CCO, used the baseline data to identify high-impact areas for process improvement. Participants in the workshop defined quality of life as a key target from the outset and brainstormed how palliative care might promote improved quality of life for patients, families, and the health care team (Fig 3). Similar to CCO, Stanford Health Care is utilizing rapid quality-improvement cycles to test new approaches. Solutions are selected based on feasibility and potential impact within the domains of primary, secondary, and specialist palliative care delivery (Table 3). Proposed solutions are being tested with disease-oriented, multidisciplinary practice teams (eg, gynecological oncology and hematology) before scaling across disease groups and the entire cancer center. Fig 3. — Word cloud illustrating responses from patients, family members, and health care professionals to the question, “How would you describe quality of life in 1 word?” Key Lessons for “Practical” Integration The PPCIP of CCO is one of the first of its kind to create a successful system for the real-time screening for palliative care needs.33 However, because of differences in culture and payment models, CCO’s processes must be modified to work within the US health care system. Thus, Stanford Health Care is Table 3. — Potential Solutions Being Piloted and Modified to Improve Quality-of-Life Care at Stanford Cancer Institute Potential Solution Description Resources Process Education Communicating Value Two questions for quality of life Oncologists are prompted to ask questions that help document goals and priorities in a patient’s health care plan. x “What to expect” symptom management and transition tool series Patients and family members are provided tailored symptom and adverse-event descriptions and management strategies at key stages along the care continuum. x x x “Here when you need me” shadow and buddy system Nurse navigators are paired with palliative care and oncology social workers for shadowing, communication role play, and ongoing “on-call” buddy support for complex needs. x x x x Palliative Care Always (online course) Oncology fellows, residents, nurse navigators, and social workers participate in a case-based, interactive course focused on patient experience, communication, and primary palliative competencies. x x x Standard screening, assessment, and referral Oncology teams co-develop a standard screening process using the PROMIS global screening and referral algorithm for supportive services. Triggers are embedded to prompt discussion of PROMIS and potential specialist support. Focus is placed on social work as in-house support. x x x Specialist “hub” with triage support for noncurative needs Specialist teams form a single service group and call center for complex needs. Screening, ad hoc oncology, and patient-initiated referrals are sent to the “hub” and connected with the appropriate specialist based on patient-centered referral criteria. x x x Core panel of quality metrics and reporting mechanism Process and outcome measures for physical, emotional, spiritual, social, cultural, and ethical domains for quality of life are regularly reported to clinical teams, patients, and administration. Results help define future modifications. x x x PROMIS = Patient Reported Outcomes Measurement Information System. 392 Cancer Control October 2015, Vol. 22, No. 4 attempting to create a modified process by learning from CCO’s best practices and allowing areas for flexibility in order to maximize the likelihood of success within a different health care system. Both organizations share common strategies for integrating palliative care. Quality Improvement Strategy CCO and Stanford Health Care employ a structured, quality-improvement process to guide the integration of palliative care into the clinical workflow. CCO leadership coached regional teams in Canada to use the Institute for Healthcare Improvement’s model to create and test concepts that might “achieve significant results in quality and innovation”; the questions utilized appear in Fig 4.40 What are we trying to accomplish? How will we know that a change is an improvement? What changes can we make that will result in improvement? Act Study Plan Do Fig 4. — Institute for Healthcare Improvement’s model for improvement leveraged by Cancer Care Ontario and Stanford Health Care. From Institute for Healthcare Improvement. Science of improvement: how to improve. http://www.ihi.org/resources/Pages/HowtoImprove/ ScienceofImprovementHowtoImprove.aspx. Reprinted with permission of the Institute for Healthcare Improvement. October 2015, Vol. 22, No. 4 The central team at CCO and Stanford Health Care developed firm aims and target metrics (question 1 and 2; see Fig 4), and then local teams were given the flexibility to develop steps that could gain traction at their own institutions and lead to the desired improvements (question 3; see Fig 4).40 The teams test potential solutions using various cycles (see Fig 4) and visible, rapid reporting mechanisms to provide feedback to stakeholders.40 In the case of CCO, immediate data availability and visibility compelled additional hospitals to join the initiative.39 Reporting also revealed opportunities for improvement.33 Stanford Health Care is starting its program with limited test pilots with single clinical teams. Data will be reported back in real time to the physicians, thus allowing for rapid learning and refining of the initial care delivery prototypes. Screening Is Necessary But Insufficient Although a standardized process for screening is necessary, resources for assessment and management are also required. CCO created a symptom management toolkit with a mobile application as a resource for health care professionals. As the screening rate edges closer to the provincial target of 70%, symptom management of severe symptoms is still inadequate.38,39 For example, patients with severe pain do not always receive an opioid.39 Thus, CCO continues to work toward improved patient symptom assessment and treatment, including strategies to modify their reporting system, to better capture how clinical teams address severe symptoms, and further engage health care professionals.39 Stanford Health Care is harnessing these insights to establish an evidence-based process for screening and is also evaluating the assessment and management processes to ensure that palliative care needs are met. Stakeholder Engagement Physician engagement was a key strategy for CCO for managing process changes throughout Ontario.33 Regional and local teams recruited self-identified clinical champions who prioritized patient experience as a philosophy of care, and these champions participated in forums to assist the executive leadership with decisions about next steps for improved care delivery and evaluation.33 Stanford Health Care is also engaging physicians, patients, and families at every level of development and testing. Patient representatives are co-developing interventions and are involved in the implementation and evaluation. Stanford Health Care has taken this 1 step forward by ensuring that patients and families are also trained in leadership skills so as to more advocate for their needs during the process. Cancer Control 393 Conclusions Although most US cancer centers report the existence of a palliative care program in their centers, palliative care remains limited to the inpatient setting in the majority of these centers.41-43 Even fewer cancer centers have integrated practices, involve palliative care in tumor boards, or embedded educational opportunities.40 Barriers to integrated practice have been described and include knowledge and attitudes regarding palliative care, limited trained workforce, and lack of care and payment models that support early and regular palliative care. The early integration of palliative care into the treatment of patients with cancer is recommended.44-46 How best to integrate palliative care into cancer care is an area of active investigation and interest. Lessons from clinical trials, as well as examples from Cancer Care Ontario and Stanford Health Care, provide strategies for scalable integration.8-10,33,38,39 These strategies include: • Create reproducible outcomes with flexible structure: Exact models for integration will evolve and vary by institution and patient population. However, even with model variability in care delivery, a consistent expectation is the early identification and assessment of palliative care needs for all patients with cancer, from the point of diagnosis through survivorship and end of life. Establishing this as a new standard of care will improve patient and family outcomes. • Start small and learn quickly: Significant opportunity lies in the principles of quality improvement and rapid learning.47 Utilizing the Institute for Healthcare Improvement approach with a set aim, clear metrics, and rapid iterative cycles is feasible and necessary to create new and successful models for integration. • Leverage primary and specialist palliative care teams: Understanding the role of primary teams for the provision of palliative care is critical for integrating palliative care into the continuum of cancer care. Providing education, training, and infrastructure to support the team’s role as palliative care providers is a promising strategy for meeting patient needs with a limited workforce. An interdisciplinary approach that includes social work and chaplaincy may further increase efficiencies in primary palliative care delivery in psychosocial and spiritual areas. • Measures matter: Current metrics utilized in palliative care studies evaluate quality of care within a narrow framework primarily limited to quality of end-of-life care and assessment of mood and physical symptoms. The development of a broad range of metrics that align with the domains of palliative care (eg, decision394 Cancer Control making, empowerment, connection) has the potential to demonstrate the anticipated increased value of integrated practice. These strategies are a starting point for institutions and health care systems to begin their own processes for integrated care. This is crucial to improve quality of care for patients with a cancer diagnosis and their families. Routinely identifying palliative care needs early on and assessing such needs both have the potential to change the conversation between patients and health care professionals and create a health care paradigm in which the values of patients and their families come front and center in the delivery of cancer care. References 1. Ingham J. The epidemiology of cancer at the end of life. In: Berger A, Porenoy RK, Weissman DE, eds. Principles and Practice of Supportive Oncology. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2002. 2. Ferris FD, Bruera E, Cherny N, et al; American Society of Clinical Oncology. Palliative cancer care a decade later: accomplishments, the need, next steps. J Clin Oncol. 2009;27(18):3052-3058. 3.American Cancer Society. Cancer Facts & Figures 2015. Atlanta, GA: American Cancer Society; 2015. http://www.cancer.org/acs /groups/content/@editorial/documents/document/acspc-044552.pdf. Accessed September 3, 2015. 4. Siegel RL, Miller KD, Jemal A. 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Cherny N, Catane R, Schrijvers D, et al. European Society for Medical Oncology (ESMO) Program for the integration of oncology and Palliative Care: a 5-year review of the Designated Centers’ incentive program. Ann Oncol. 2010;21(2):362-369. 16. American Society of Clinical Oncology. Cancer care during the last phase of life. J Clin Oncol. 1998;16(5):1986-1996. 17. Davis MP, Strasser F, Cherny N. How well is palliative care integrated into cancer care? A MASCC, ESMO, and EAPC Project. Support Care Cancer. 2015;23(9):2677-85. 18. Gelfman LP, Meier DE, Morrison RS. Does palliative care improve quality? A survey of bereaved family members. J Pain Symptom Manage. 2008;36(1):22-28. 19. Hui D, Kim YJ, Park JC, et al. Integration of oncology and palliative care: a systematic review. Oncologist. 2015;20(1):77-83. 20. Brumley R, Enguidanos S, Jamison P, et al. Increased satisfaction October 2015, Vol. 22, No. 4 with care and lower costs: results of a randomized trial of in-home palliative care. J Am Geriatr Soc. 2007;55(7):993-1000. 21. Gade G, Venohr I, Conner D, et al. Impact of an inpatient palliative care team: a randomized control trial. J Palliat Med. 2008;11(2):180-190. 22. Pantilat SZ, O’Riordan DL, Dibble SL, et al. Hospital-based palliative medicine consultation: a randomized controlled trial. Arch Int Med. 2010;170(22):2038-2040. 23. Center to Advance Palliative Care. 2011 Public Opinion Research on Palliative Care: A Report Based on Research by Public Opinion Strategies. New York: Center to Advance Palliative Care; 2011. https://media.capc.org/filer_public/18/ab/18ab708c-f835-4380-921dfbf729702e36/2011-public-opinion-research-on-palliative-care.pdf. Accessed September 3, 2015. 24. Schenker Y, Park SY, Maciasz R, et al. Do patients with advanced cancer and unmet palliative care needs have an interest in receiving palliative care services? J Palliat Med. 2014;17(6):667-762. 25. Schenker Y, Crowley-Matoka M, Dohan D, et al. Oncologist factors that influence referrals to subspecialty palliative care clinics. J Oncol Pract. 2014;10(2):e37-e44. 26. Irwin KE, Greer JA, Khatib J, et al. Early palliative care and metastatic non-small cell lung cancer: potential mechanisms of prolonged survival. Chron Respir Dis. 2013;10(1):35-47. 27. von Gunten CF. Secondary and tertiary palliative care in US hospitals. JAMA. 2002;287(7):875-881. 28. Kamal AH, Gradison M, Maguire JM, et al. Quality measures for palliative care in patients with cancer: a systematic review. J Oncol Pract. 2014;10(4):281-287. 29. Ramsey S, Schickedanz A. How should we define value in cancer care? Oncologist. 2010;15(suppl 1):1-4. 30. Neuss MN, Desch CE, McNiff KK, et al. A process for measuring the quality of cancer care: the Quality Oncology Practice Initiative. J Clin Oncol. 2005;23(25):6233-6239. 31. Weissman DE, Meier DE. Identifying patients in need of a palliative care assessment in the hospital setting: a consensus report from the Center to Advance Palliative Care. J Palliat Med. 2011;14(1):17-23. 32. Temel JS, Greer JA, Admane S, et al. Longitudinal perceptions of prognosis and goals of therapy in patients with metastatic non-small-cell lung cancer: results of a randomized study of early palliative care. J Clin Oncol. 2011;29(17):2319-2326. 33. Dudgeon D, King S, Howell D, et al. Cancer Care Ontario’s experience with implementation of routine physical and psychological symptom distress screening. Psychooncology. 2012;21(4):357-364. 34. Hydeman J. Improving the integration of palliative care in a comprehensive oncology center: increasing primary care referrals to palliative care. Omega (Westport). 2013;67(1-2):127-134. 35. Dyar S, Lesperance M, Shannon R, et al. A nurse practitioner directed intervention improves the quality of life of patients with metastatic cancer: results of a randomized pilot study. J Palliat Med. 2012;15(8):890-895. 36. Glare PA, Semple D, Stabler SM, et al. Palliative care in the outpatient oncology setting: evaluation of a practical set of referral criteria. J Oncol Pract. 2011;7(6):366-370. 37. Stanford Medicine website. Stanford Medicine initiatives. https:// med.stanford.edu/about/vision/initiatives.html. Accessed September 3, 2015. 38. Cancer Care Ontario website. https://www.cancercare.on.ca. Accessed September 11, 2015. 39. Pereira J, Green E, Molloy S, et al. Population-based standardized symptom screening: Cancer Care Ontario’s Edmonton Symptom Assessment System and performance status initiatives. J Oncol Pract. 2014;10(3):212-214. 40. Institute for Healthcare Improvement. Science of improvement: how to improve. http://www.ihi.org/resources/Pages/HowtoImprove/ScienceofImprovementHowtoImprove.aspx. Accessed September 3, 2015. 41. Hui D, Elsayem A, De la Cruz M, et al. Availability and integration of palliative care at US cancer centers. JAMA. 2010;303(11):1054-1061. 42. Goldsmith B, Dietrich J, Du Q, et al. Variability in access to hospital palliative care in the United States. J Palliat Med. 2008;11(8):1094-1102. 43. Morrison RS, Augustin R, Souvanna P, et al. America’s care of serious illness: a state-by-state report card on access to palliative care in our nation’s hospitals. J Palliat Med. 2011;14(10):1094-1096. 44. Hui D, Elsayem A, De la Cruz M, et al. Availability and integration of palliative care at US cancer centers. JAMA. 2010;303(11):1054-1061. 45. Gelfman LP, Morrison RS. Research funding for palliative medicine. J Palliat Med. 2008;11(1):36-43. 46. Abrahm JL. Integrating palliative care into comprehensive cancer care. J Natl Compr Canc Netw. 2012;10(10):1192-1198. 47. Kamal AH, Miriovsky BJ, Currow DC, et al. Improving the management of dyspnea in the community using rapid learning approaches. Chron Respir Dis. 2012;9(1):51-61. October 2015, Vol. 22, No. 4 Cancer Control 395 Quality-improvement collaborations will continue to grow in the field of palliative care. Photo courtesy of Lisa Scholder. Road Between Art, 16" × 24". Improving the Quality of Palliative Care Through National and Regional Collaboration Efforts Arif H. Kamal, MD, MHS, Krista L. Harrison, PhD, Marie Bakitas, DNSc, CRNP, J. Nicholas DionneOdom, PhD, Lisa Zubkoff, PhD, Imatullah Akyar, PhD, RN, Steven Z. Pantilat, MD, David L O’Riordan, PhD, Ashley R. Bragg, Kara E. Bischoff, MD, and Janet Bull, MD Background: The measurement and reporting of the quality of care in the field of palliation has become a required task for many health care leaders and specialists in palliative care. Such efforts are aided when organizations collaborate together to share lessons learned. Methods: The authors reviewed examples of quality-improvement collaborations in palliative care to understand the similarities, differences, and future directions of quality measurement and improvement strategies in the discipline. Results: Three examples were identified that showed areas of robust and growing quality-improvement collaboration in the field of palliative care: the Global Palliative Care Quality Alliance, Palliative Care Quality Network, and Project Educate, Nurture, Advise, Before Life Ends. These efforts exemplify how shared-improvement activities can inform improved practice for organizations participating in collaboration. Conclusions: National and regional collaboratives can be used to enhance the quality of palliative care and are important efforts to standardize and improve the delivery of palliative care for persons with serious illness, along with their friends, family, and caregivers. From the Duke Cancer Institute, Durham, North Carolina, Capital Caring (KLH), Falls Church, Virginia, School of Nursing (MB, JND-O, IA), University of Alabama at Birmingham, Birmingham, Alabama, White River Junction Veterans Affairs Medical Center (LZ), White River Junction, Vermont, Palliative Care Program (SZP, DLO, ARB, KEB), University of California, San Francisco, California, and Four Seasons Compassion for Life (JB), Flat Rock, North Carolina. Submitted May 12, 2015; accepted July 30, 2015. Address correspondence to Arif H. Kamal, MD, MHS, Duke Cancer Institute, 2400 Pratt Street, Durham, NC 27710. E-mail: arif.kamal@duke.edu Dr Harrison is an employee of Capital Care and was involved with the Global Palliative Care Quality Alliance and Palliative Care Research Cooperative Group during the writing of this manuscript. Dr Bull is an advisor for Aspire Health. No significant relationships exist between Ms Bakitas and Bragg or Drs Kamal, Akyar, Dionne-Odom, Zubkoff, O’Riordan, and Bischoff and the companies/organizations whose products or services may be referenced in this article. 396 Cancer Control Introduction Over the last few years, use and acceptance of palliative care have been growing for the complex care and needs of patients with cancer and their loved ones.1 This reflects an increased understanding and acceptance of the care philosophy, the value of interdisciplinary teams that focus on the patient and his or her family as the unit of care, and the understanding that the goals of palliative care align with the priorities of all stakeholders.2 More patients are receiving palliative care because the scope of practice has expanded from community-based hospices to inpatient hospitals to the offices of primary care physicians, and the number of health care professionals seeking specialized continuing education and support to improve palliative care has increased.3 In parallel with this clinical growth, October 2015, Vol. 22, No. 4 collaborations to improve the quality of palliative care are also becoming more common, aiming to share data across sites and settings to translate quality improvement activities across the discipline.4 Quality-improvement collaborations aim to clarify how care is delivered and how care might be enhanced through realistic and sustainable interventions. The challenges to such collaborations vary by type and setting of practice, but capturing the diversity of care-delivery systems is paramount to understanding the variation of palliative care in settings, ranging from community-based organizations to large, academic medical centers. Collaborative initiatives to study the quality of care reflect our increasing reliance on data collection and analysis to understand broader patterns across health care, whether for program development, reimbursement, health services research, or quality assessment. Thus, this article will focus on 3 efforts to improve the quality of the delivery of palliative care at regional and national levels. Global Palliative Care Quality Alliance The Global Palliative Care Quality Alliance (GPCQA) is a novel, community/academic quality assessment and improvement collaboration for consultative palliative care that aims to5: • Collect data on the quality of care delivery using a standardized, point-of-care delivery approach • Facilitate conversations between practices of all sizes and locations regarding challenges and sustainable solutions to improve care • Use a Institute of Medicine–recommended rapidlearning health care approach to simultaneously inform clinical care, quality measurement, and outcomes research • Monitor patients along the continuum of care • Allow for benchmarking and sharing of best practices The GPCQA originally began in 2007 as the Carolinas Consortium for Palliative Care. Realizing the need to test and adopt this model of quality improvement, its founders set out after initial planning in 2005 to establish an academic/community collaboration between Duke University (Durham, North Carolina) and community partners. The Carolinas Consortium for Palliative Care was originally composed of 5 sites throughout North Carolina: Duke University Medical Center (Durham) and 4 palliative care organizations, namely Four Seasons Compassion for Life (Flat Rock), Forsyth Palliative Care (Winston-Salem), Hospice of Wake and Horizons Palliative Care (Raleigh), and Charlotte Hospice and Palliative Care (Charlotte). Data from patients were collected by health care professionals near the point of care, entered into a local database, and intermittently transmitted to a centralized dataset maintained at Duke University for analysis and quality reOctober 2015, Vol. 22, No. 4 porting. The information contributed to a growing data resource, the palliative care database (PCD), which is used for quality assessment and research purposes. From 2008 to 2011, a total of 6,957 unique patient data were collected. Data analyses also supported organizational, quality-improvement objectives and descriptive research about the population served by the Carolinas Consortium for Palliative Care, providing proof of concept that collecting data on quality is feasible in community settings and that these data can inform both clinical practice and institutional priorities in community-based palliative care.6 However, data collection processes were inefficient and the data collected did not always map to emerging quality measures. Further, a need exists to expand beyond the Carolinas to include partners from across the United States and the world so as to represent greater diversity in practice and patient needs. A contemporary-based solution was needed that could be electronically implemented in various institutions outside the region.7 In 2012, the Quality Data Collection Tool (QDACT) for palliative care was created, leveraging an iterative design in which modular components could be changed as quality measures in palliative care evolved. To develop a quality-assessment tool applicable to everyday practice, 6 steps were involved. The first step was to review the experiences from participants of the PCD project.6 This proof-of-concept pilot confirmed that collecting data on quality was feasible in community settings.5 The second step was to perform a systematic review of all published quality measures relevant to palliative care, supportive oncology, and end-of-life care to identify measures from which the Consortium could choose to establish priorities for assessment.8 The third step was to develop a list of validated tools from a literature review that would inform these quality measures. We aimed to incorporate tools familiar to palliative care providers, when available, like the Palliative Performance Scale. In some instances, the Carolinas Consortium for Palliative Care added metrics and associated data elements based on group consensus. The fourth step involved developing QDACT, an instrument that demonstrated scalability across expected future changes in the collection and sharing of data relating to palliative care. Electronic health systems and platforms for collecting data evolve and change, so it was important that the tool be generic and operable on different — rather than specific — operating systems, computer hardware, or Internet platforms and be compatible with diverse information technology resources used by palliative care programs nationwide. This process included the development of a data dictionary to support the quality measures. The fifth step was to test the entire process, from Cancer Control 397 data collection to transmission, storage, analysis, and management; in addition, the process was tested to ensure that it conformed to the highest data security standards for protected health information, including those of the Health Insurance Portability and Accountability Act. This included thorough understanding of the challenges to data security that stem from both hardware and software used at the point of care as well as the potential risks of transmitting data over diverse networks to a shared database. The sixth step was to expand the project and test its usability on a national scale. The Carolinas Consortium for Palliative Care was renamed GPCQA in 2014, and it has 14 members who represent community and academic settings and span outpatient and inpatient palliative care programs. Reporting on quality metrics is performed each quarter for all sites, with a feedback loop to improve graphics and visual features. More than 33,000 patient encounters are represented in the database. The quality reporting has been used to support grant funding, to demonstrate the value of palliative care to hospitals and partnering organizations, to understand the patient population served at each participating institution, and to inform quality-improvement projects. QDACT displays real-time, immediate feedback via a color-coded alert system while having built-in logic to help inform clinical decisions while health care professionals enter data. For example, a color-coded system reflects whether responses meet an alarm threshold, which is an evidence- or consensus-based parameter (eg, pain score > 4 out of 10). At that point, a screen opens to prompt the user to input what treatments have been offered, if any, and a list of available evidence-based options are displayed. In this example, the alert color (red) will remain present until the pain is brought below the threshold. Other aggregate reports include longitudinal summaries customizable to the health care professional and his or her organization. Members of the GPCQA can request reports that provide both numerical and graphical presentations of descriptive statistics on patient needs, conformance to quality measures, comparative performance between reporting levels, and longitudinal changes. Reports under development include those aligned to national quality measurement initiatives (eg, National Quality Forum, American Academy of Hospice and Palliative Medicine’s Measuring What Matters, Physician Quality Reporting Structure), financial projections, and costavoidance reports. This infrastructure has been used to conduct several quality-improvement projects. One project simultaneously measured and assessed adherence to quality measures for timely assessment and management of pain, dyspnea, and constipation. Using reports tailored to the performance of individual health care profes398 Cancer Control sionals along with peer-related benchmarks, and then confidentially sharing those reports with those health care professionals, we observed an improvement of the timely management of these 3 important symptoms to levels above 95%. Longitudinal data collection will inform whether these results are long lasting. Another quality-improvement project involved assessing spirituality and existential distress during the first palliative care visit in all care settings. Using a goal of more than 75% for the completion of the spiritual assessment, the results of the project demonstrated 80% compliance with completing the spirituality question across all of the providers within the GPCQA. The GPCQA is also looking to expand its membership. Further plans include customized modular build-outs as part of QDACT, expansion of reporting features, integration with electronic health records (EHRs), introduction of a mobile application, and features that enable programs to pull data directly from the software platform. As the landscape of quality measure reporting increases in an era of value-based health care delivery, the GPCQA will look to continuously evolve its efforts to minimize the burden of data collection by health care professionals while maximizing the return on investment to health care professionals through reports, information to support program growth and sustainability, and financial benefits that reward health care professionals for actively promoting a culture of quality assessment and improvement. Palliative Care Quality Network The Palliative Care Quality Network (PCQN) is a continuous learning collaborative committed to improving the quality of palliative care services provided to patients and their families. The PCQN was established in 2009 by 20 established palliative care services in California with a shared vision for improving the quality and value of care. The PCQN developed a core dataset that includes care processes (eg, psychosocial assessments) and patient level outcomes (eg, symptom scores) that all members collect on every patient visit. Data are entered into the secure, web-based PCQN database that analyzes data and produces custom reports in real time for individual teams, with comparisons to the entire PCQN. PCQN data allow for coordinated, quality-improvement projects, benchmarking, and the identification of best practices. The PCQN is composed of 34 palliative care teams from hospitals across the United States and includes community, academic, and public institutions. To date, 19 palliative care teams collect and submit data to the PCQN database and use those data to monitor and improve care. The PCQN database includes more than 13,000 patient encounters. An important goal of the PCQN is to create and foster a professional community that contributes to the October 2015, Vol. 22, No. 4 growth and sustainability of palliative care teams and the professional development of the health care professionals that staff them. The PCQN achieves this goal by providing education in clinical care, leadership, team dynamics, and self-care, creating a forum for members to share successful strategies, offering a software program to calculate the financial impact of palliative care at each institution, and establishing a supportive network of like-minded health care professionals. Guided by the goals of the prospective collection of operational and clinical outcomes data to support real-time patient care and quality improvement, efficiency, and adherence to national guidelines, including the NCP and NQF, the PCQN undertook a modified Delphi process to develop the PCQN core dataset. Overall, the 20 founding palliative care service members collected 96 unique data elements. Through a survey of all members, the PCQN identified 48 “must collect” elements. The PCQN data committee evaluated these elements against published preferred practices to create a 23-item core dataset that includes demographics, processes of care, and patient-level clinical outcomes, including daily symptom scores. The data committee also created a data dictionary to define each data element to ensure consistency across health care professionals and care sites. A key goal of the PCQN was that the database would include all data that teams needed to collect; no PCQN member would need to maintain a secondary database. To that end, the PCQN adopted optional data elements requested by individual teams for monitoring care at their site. To date, the PCQN has added 22 optional data elements. The PCQN then developed a secure, web-based database for convenient data entry. PCQN members directly enter data at the point of care into any Internet-connected device or on paper for later data entry. Teams can also collect PCQN data in the EHR, download those data to a spreadsheet file and then upload the file to the PCQN. When data are entered into the PCQN database, they are included in analyses. The PCQN is collaborating with teams using several EHR platforms to fully integrate the collection of these data. Each member organization owns its data and can download all of the data at any time to a spreadsheet. Data are confidential; only member organizations know which data are theirs. The PCQN database performs analyses that compare data from the entire network. Members can generate 4 types of summary reports in real time, including reports on demographics, processes of care, disposition and length of stay, and symptoms. Trend reports of data over time and member-comparison reports show the data of the member organization highlighted alongside the unidentified data of the other member organizations. The PCQN is also creating reOctober 2015, Vol. 22, No. 4 ports that will allow members to compare themselves with others in their health care system. In addition, members can submit information about the structure of their team and characteristics of their institution to compare themselves with similar teams as well as to the entire database. The PCQN database also produces reports on data completeness and identifies missing data so that members can monitor data-collection efforts. PCQN data align with the Measuring What Matters measures of the American Academy of Hospice and Palliative Medicine/Hospice and Palliative Nurses Association have been used by members to achieve The Joint Commission advanced certification in palliative care.9 The PCQN has developed a financial software program, CaseMaker PCS (San Francisco, California), to calculate the financial outcomes of palliative care at each member institution. CaseMaker PCS combines PCQN data with financial data supplied by member institutions to produce an editable summary of the financial impact of the palliative care team’s work. Combined with outcomes data demonstrating quality and comparative data on team composition, the financial analysis information provides palliative care teams information needed to demonstrate value and proof for sustaining and growing their service. A PCQN quality-improvement collaborative was launched in 2014 with the goals of providing education in quality-improvement methods and using PCQN data to drive coordinated quality-improvement projects. Through quality-improvement workshops at biannual conferences, monthly conference calls, and ongoing mentoring, teams in the collaborative share strategies, challenges, and successes to advance a quality-improvement project. Nine PCQN teams are participating in the first PCQN quality-improvement project, which is focused on improving pain management (a patientreported outcome). PCQN data show that, at baseline, 69% (range, 62%–80%) of patients with moderate or severe pain on the day of their initial palliative care assessment had an improvement in their pain by the second palliative care assessment, but only when that assessment occurred within 72 hours of the first. Through the quality-improvement collaborative, participants have identified and tested a number of strategies aimed at improving pain management, including seeing patients with pain early in the day, contacting primary teams and nurses with recommendations or writing new orders immediately after seeing a patient in pain, and conducting follow-up visits in the afternoon to reassess pain and amend recommendations or orders. Members are monitoring these processes as well as their associated daily pain scores to determine the success of each test of change. To date, improvements have been difficult to Cancer Control 399 achieve, but the variation in performance is helping to identify additional targets, including the value of addressing anxiety in patients with pain, and the project is ongoing. Members with better outcomes share strategies with others on each monthly conference call, struggling members are provided ideas about how to overcome stumbling blocks, and regular calls motivate all members to keep the project active and advancing. Satisfaction with the quality-improvement collaborative is high, with 82% of respondents agreeing that their participation motivated their member organization to engage in quality improvement, and 83% indicating that they were interested in continuing their participation in the quality-improvement collaborative. The PCQN is growing and is inviting additional palliative care teams to join. The more members, the greater the capacity to benchmark with like hospitals to determine with greater precision which structures and processes of care are associated with better outcomes. The PCQN is developing outpatient and homebased palliative care datasets to link with the existing PCQN database, allowing members to monitor and improve care across settings and over time. The PCQN is also working with vendors of EHRs to create systems for quality data collection within the EHR to be submitted to the PCQN database. The PCQN continues to refine its approach to quality improvement and will engage in additional quality-improvement projects involving more teams. Improving palliative care is a key goal and, thus, a broad dissemination of effective strategies to improve care is a major focus of PCQN going forward. The PCQN is partnering with other organizations and quality initiatives within and outside of palliative care, including the GPCQA, to improve the quality of care across the field, with the mission of transforming health care by defining and promoting quality palliative care. Project Educate, Nurture, Advise, Before Life Ends Project Educate, Nurture, Advise, Before Life Ends (ENABLE) is an evidence-based, telehealth, upstream, palliative care model designed to improve care and quality of life for persons with newly diagnosed advanced cancer.10 First developed in 1999 as a demonstration project through the Robert Wood Johnson Foundation, Project ENABLE has undergone efficacy and effectiveness testing through 2 randomized controlled trials funded by the National Institutes of Health.10-13 Because of its demonstrated benefits to patient quality of life, symptoms, mood, rates of survival, and to family caregiver depression and burden, Project ENABLE is a scalable model of care.14-16 The project was developed and tested in primarily rural, community-based cancer practices; however, little guidance exists on how com400 Cancer Control munity cancer centers can integrate this model of early concurrent palliative care alongside standard, curative cancer therapies. In 2013, through a research scholar grant from the American Cancer Society, we embarked on a 4-year dissemination/implementation project using a virtuallearning collaborative approach in 4 racially diverse settings. The project goals were to: • Assess current palliative care practices and prepare for organizational/health care system change • Tailor and implement the evidence-based, concurrent, palliative care model of Project ENABLE • Use the Reach, Effectiveness, Adoption, Implementation, and Maintenance (RE-AIM) framework to assess outcomes associated with health care institutions, patients and caregivers, and costs before and after implementation of the model We chose a virtual-learning collaborative approach for 3 reasons because we wanted to increase access to nationwide sites in predominantly rural areas, examine the effectiveness and scalability of this model in racially and ethnically diverse settings, and modify and hone implementation strategies for future dissemination of the program. In addition, we chose RE-AIM because it is a well-tested framework to evaluate the impact of new public health programs.17 The first step was to establish working relationships and procedures to launch a virtual collaborative between the School of Nursing at the University of Alabama at Birmingham, which was the coordinating center, and the 4 sites (Gibbs Cancer Center [Spartanburg, South Carolina], Mitchel Cancer Center [Mobile, Alabama], Birmingham Veterans Administration Medical Center [Birmingham], and the Department of Gynecologic Oncology, Wallace Tumor Institute, University of Alabama at Birmingham). Over the last 2 years, the teams at each institution met every 2 weeks as a group with the coordinating center via video conferencing using an online learning platform. During the intervening weeks, the coordinating center team met to trouble-shoot, develop, and refine processes. Early steps included defining the essential elements of the Project ENABLE model and working with individual sites to tailor the model’s elements to specific institutional cultures and resources. Through dialogue and consensus with sites, we also established implementation measures guided by the RE-AIM framework. However, because RE-AIM was not previously used in the setting of palliative care or oncology, time was spent adapting the RE-AIM framework to suit our model and the population of newly diagnosed patients with advanced cancer and their caregivers. To measure the capacity of organizational characteristics to implement and sustain Project ENABLE, we adapted the General Organizational Index scale.18 Data collection included a survey to measure the perOctober 2015, Vol. 22, No. 4 ceptions of oncologists about early and concurrent palliative care, a tool to examine implementation costs, and a battery of patient and caregiver self-report measures similar to those used in our prior randomized controlled trials.11,12 Mixed methods were used to collect data, including in-person site visit interviews, webbased surveys, and phone interviews. We have experienced challenges and successes related to the implementation of this model, and we have learned lessons along the way. For example, each site received a small yearly stipend to offset some initial implementation costs (eg, developing materials, fees related to Institutional Review Boards, salary support for conducting the program), but this stipend was not intended to fully support a new program. As such, institutions have had to envision strategies for supporting their programs beyond the grant period. As a result of changes in institutional leadership, 2 sites were unable to continue and the 2 replacement sites had to quickly acclimate to the model during the planning year. Implementation studies are uncommon to many Institutional Review Boards, so there were many delays prior to acquiring Institutional Review Board approval in the 4 diverse community institutions (including 1 Veterans Administration medical center).19 One success included 2 functioning programs that introduced potentially sustainable models of early palliative care concurrent with standard cancer treatment, thus increasing access, as was defined in the original project aims. Another success was identifying promising implementation strategies (guided by the RE-AIM framework) for developing new palliative care programs. The important lessons learned, such as institutional barriers and facilitators of implementation, will serve as a foundation for future progress. Our future goals within the context of this project are to refine all processes and measures to create a toolkit that could be used by other centers wishing to implement an early palliative care model, either in settings with existing palliative care teams without an outpatient or home-/community-based component or within systems without a functional palliative care model. In addition, we will use the current model and methods as pilot data for a larger implementation grant within an established practice network, such as across the US Veterans Administration health care systems, the Deep South Network for Cancer Control, or a research network such as the Palliative Care Research Cooperative Group. Discussion Quality-improvement initiatives reflect the natural evolution of the evidence base in palliative care. Use of palliative care has grown in the last decade, with its roots beginning in the hospice movement in the late 1970s. A defined subspecialty field, with an eviOctober 2015, Vol. 22, No. 4 dence base for practice that improves daily palliative care, should use implementation and dissemination approaches that routinely include elements of quality measurement (often with validated measurement tools). Federated database systems facilitate the simultaneous examination of both quality and research questions. Administrators, health care professionals, and researchers are beginning to recognize that robust data analytics are crucial for improving patient- and family-centered advanced illness care. The 3 quality-improvement and dissemination efforts reviewed in this article all use a conceptual model of quality improvement in palliative care to guide their approach, highlighting the importance of collecting uniform data. For example, all 3 efforts require participants to use a federated set of primary data elements defined by a common database dictionary as part of the validated collection metrics. Given the variety of practice settings, clinician types, and stages of disease process represented, this allows for uniformity and interoperability that ensures that data can be aggregated, compared, and analyzed in the future to better characterize palliative care. One significant, ongoing challenge for new and existing quality-improvement initiatives is how to make the effort as invisible and integrated as possible amid the fabric of usual clinical operations. As the GPCQA has found, the dual-entry of data elements — once into the EHR for clinical purposes, and once into a registry for aggregation and reporting — is a significant barrier to health care professional buy-in and effectiveness. The seamless integration of validated, quality metric data collection into EHRs is ideal but has major cost and time implications. However, overcoming these barriers is crucial to allow for increasingly robust reporting of data, which can then be used to aid palliative care program development, sustainability, and growth. Future Directions Quality-improvement collaborations are expected to continue to grow in the field of palliative care. For established efforts such as the 3 described in this article, this growth will manifest as a focus on how to sustain the initiative in terms of mutually beneficial outcomes, continued innovation, and financial viability. In turn, sustainability will allow for additional innovation, such as developing quality-improvement initiatives focused on patient-reported outcomes and caregiver outcomes. Programs may join forces and align with other initiatives to allow for larger learning networks and more robust data. By contrast, small, issue-specific collaborations may develop where practices are less unified, such as when palliative care is incorporated into alternative payment model structures (eg, medical homes, accountable care orCancer Control 401 ganizations). Similarly, local efforts may develop among health care professionals in a single region so that a single patient’s trajectory can be tracked — and quality of palliative care improved — across multiple care transitions and health care systems. As the push for meaningful use and interoperability of electronic health records continues, uniform and secure data collection and sharing (per the requirements of the Health Insurance Portability and Accountability Act) should continue to improve. Conclusions Health care leaders and those specializing in palliative care are faced with the challenge of providing consistent, high-quality care that meets the needs of patients and their family, friends, and caregivers. This challenge can be aided by belonging to a community of like-minded leaders focused on cooperative efforts to define, measure, and improve the quality of palliative care delivery. Clinical palliative care is a collaborative effort, so alliances and networks of engaged health care leaders and physicians are needed to prioritize the care processes that consistently improve the experiences of patients with serious illnesses. versus delayed palliative care to informal family caregivers of patients with advanced cancer: outcomes from the ENABLE III randomized controlled trial. J Clin Oncol. 2015;33(13):1446-1452. 14. Gomes B, Calanzani N, Curiale V, et al. Effectiveness and cost-effectiveness of home palliative care services for adults with advanced illness and their caregivers. Cochrane Database Syst Rev. 2013;6:CD007760. 15. Smith TJ, Temin S, Alesi ER, et al. American Society of Clinical Oncology provisional clinical opinion: the integration of palliative care into standard oncology care. J Clin Oncol. 2012;30(8):880-887. 16. National Cancer Institute. Research-tested intervention programs: Project ENABLE II. Updated September 25, 2014. http://rtips.cancer.gov /rtips/programDetails.do?programId=3590238. Accessed September 10, 2015. 17. Glasgow RE, Vogt TM, Boles SM. Evaluating the public health impact of health promotion interventions: the RE-AIM framework. Am J Public Health. 1999;89(9):1322-1327. 18. McGovern MP, McHugo GJ, Drake RE, et al. Implementing EvidenceBased Practices in Behavioral Health. Center City, MN: Hazelden; 2013. 19. Patel DI, Stevens KR, Puga F. Variations in institutional review board approval in the implementation of an improvement research study. Nurs Res Pract. 2013;2013:548591. Acknowledgment: The authors would like to recognize the dedication of our colleague clinicians and patients who make this work possible. References 1.Center to Advance Palliative Care; National Palliative Care Registry; National Palliative Care Research Center. National Palliative Care Registry Annual Survey Summary: Results of the 2012 National Palliative Care Registry Survey, as of July 2014. 2014. https://registry.capc.org/cms /por tals/1/Repor ts/National_ Palliative_Care%20Registr y _ Annual_ Survey_Summary_9.2.2014.pdf. Accessed September 15, 2015. 2. Swetz KM, Kamal AH. In the clinic. Palliative care. Ann Intern Med. 2012;156(3):ITC2-1, TC2-2-TC2-15. 3. Kamal AH, Currow DC, Ritchie CS, et al. Community-based palliative care: the natural evolution for palliative care delivery in the U.S. J Pain Symptom Manage. 2013;46(2):254-264. 4. Kamal AH, Hanson LC, Casarett DJ, et al. The quality imperative for palliative care. J Pain Symptom Manage. 2015;49(2):243-253. 5. Kamal AH, Kavalieratos D, Bull J, et al. Usability and acceptability of the QDACT-PC, an electronic point-of-care system for standardized quality monitoring in palliative care. J Pain Symptom Manage. 2015;50(5): 615-621. 6. Kamal AH, Bull J, Stinson C, et al. Collecting data on quality is feasible in community-based palliative care. J Pain Symptom Manage. 2011;42(5):663-667. 7. Kamal AH, Swetz KM, Dy S, et al. Integrating technology into palliative care research. Curr Opin Support Palliat Care. 2012;6(4):525-532. 8. Kamal AH, Gradison M, Maguire JM, et al. Quality measures for palliative care in patients with cancer: a systematic review. J Oncol Pract. 2014;10(4):281-287. 9. Dy SM, Kiley KB, Ast K, et al. Measuring what matters: top-ranked quality indicators for hospice and palliative care from the American Academy of Hospice and Palliative Medicine and Hospice and Palliative Nurses Association. J Pain Symptom Manage. 2015;49(4):773-781. 10. Bakitas M, Stevens M, Ahles T, et al; Project Enable Co-Investigators. Project ENABLE: a palliative care demonstration project for advanced cancer patients in three settings. J Palliat Med. 2004;7(2):363-372. 11. Bakitas M, Lyons KD, Hegel MT, et al. Effects of a palliative care intervention on clinical outcomes in patients with advanced cancer: the Project ENABLE II randomized controlled trial. JAMA. 2009;302(7):741-749. 12. Bakitas MA, Tosteson TD, Li Z, et al. Early versus delayed initiation of concurrent palliative oncology care: patient outcomes in the ENABLE III randomized controlled trial. J Clin Oncol. 2015;33(13):1438-1445. 13. Dionne-Odom JN, Azuero A, Lyons KD, et al. Benefits of early 402 Cancer Control October 2015, Vol. 22, No. 4 A systematic approach to managing symptoms related to advanced oncological illness can improve patient quality of life and lessen distress. Lisa Scholder. Sunlit Legs. 16" × 22". Photo courtesy of Lisa Scholder. Palliative Pharmacotherapy: State-of-the-Art Management of Symptoms in Patients With Cancer Eric E. Prommer, MD Background: Advanced cancer produces multiple symptoms as patients progress through their disease trajectory. Identifying, measuring, and providing therapy for uncontrolled symptoms becomes important because disease-altering therapies may be no longer possible. Symptoms other than pain that cause distress in patients with cancer include delirium, dyspnea, anorexia, nausea, and fatigue. Precise management of these symptoms can lead to the best possible quality of life and lessen distress. This article reviews current management strategies of these symptoms. Methods: The epidemiology, mechanisms, assessment, and therapies of common symptoms in the advanced cancer population are reviewed. Results: Identifiable approaches facilitate symptom management in advanced illness. Conclusions: Using a systematic approach to symptoms in advanced illness can improve the quality of life and lessen distress among patients with cancer and their families, friends, and caregivers. Delirium Delirium is a clinical emergency characterized by changes in consciousness, hallucinations, and changes in the sleep–wake cycle and language. Delirium is a frequent event in patients with advanced cancer, and factors predisposing patients with advanced cancer include drugs, infection, brain metastasis, and underlying dementia.1 Delirium differs from dementia in that dementia does not have acute alterations From the Division of Hematology/Oncology, Veterans Integrated Palliative Care Program, Veterans Integrated Palliative Care, David Geffen School of Medicine, University of California, Los Angeles, California. Submitted July 30, 2015; accepted September 16, 2015. Address correspondence to Eric E. Prommer, MD, David Geffen School of Medicine, University of California, 11301 Wilshire Boulevard, Building 500, Room 2064A, Mail Code 10P, Los Angeles, CA 90073. E-mail: Eric.prommer@va.gov No significant relationship exists between the author and the companies/organizations whose products or services may be referenced in this article. October 2015, Vol. 22, No. 4 in consciousness.2 Delirium is classified according to level of agitation; for example, a patient who is agitated has hyperactive delirium and a patient who is withdrawn and somnolent has hypoactive delirium.3 Patients usually have mixed features. Prevalence rates for delirium range from 30% to 50% for hospitalized patients and is typical in the hours or days before death.4 Experiencing delirium is likely to cause distress in families.5 Patients who are delirious are unable to communicate levels of pain or other symptoms and cannot take part in health care decisions.6 Although delirium is often a harbinger of a poor prognosis, it is reversible in 50% of cases.7 Health care professionals use nonpharmacological as well as pharmacological interventions to treat delirium. Nonpharmacological approaches to delirium include using clocks to show the time, lighting the room, and frequent reorientation, whereas pharmacological interventions may include the use of antipsychotic medications, which can be used to palliate Cancer Control 403 patients who are agitated (hyperactive delirium) as well as those experiencing hallucinations regardless of agitation level. Mechanisms Delirium results from underlying disorders that cause imbalances in brain neurotransmitters. Neurotransmitters involved in delirium include dopamine, glutamate, norepinephrine, acetylcholine, g-aminobutyric acid, and serotonin.8 Cytokines (interleukin [IL]-1, IL-2, tumor necrosis factor [TNF], interferon) produced by the immune system, the tumor, or cancer treatment may mediate central nervous system effects, such as somnolence, agitation, and cognitive failure.9 Drug therapies for delirium target imbalances in neurotransmitters, which may play a role in developing delirium. Assessment A history of the patient’s baseline mental status prior to symptom onset should be obtained from his or her family, caregivers, or both parties. Fluctuating consciousness is the hallmark of delirium. Assessment tools can screen or rate delirium or do both. Commonly used tools include those for screening (Mini Mental State Examination, Confusion Assessment Method) or rating of severity (Memorial Delirium Assessment Scale, Disability Rating Scale). Some instruments will address more than 1 goal. Other important considerations are time constraints, the level of expertise and training of the investigator (the Mini Mental State Examination requires none), and constraints of the patient (eg, patient in the intensive care unit).10 Management Nonpharmacological: Identification of reversible causes is important. Investigations should be tailored to each individual patient’s goals of care because 50% of cases of delirium may be reversible.7 Health care professionals must look for infection, dehydration, and drug and metabolic abnormalities as potentially reversible causes of delirium. Patients with cancer should be evaluated for central nervous system metastasis. It is worth noting that fecal impaction and urinary tract infection are often overlooked as causes of delirium. Common drugs linked to delirium include opioids, anticholinergics, benzodiazepines steroids, and select chemotherapy agents. Inquiring about alcohol intake is prudent because alcohol withdrawal can precipitate delirium and responds to benzodiazepines. Nonpharmacological approaches to delirium include keeping room lights on, having calendars and pictures at the bedside, frequent redirection, and allowing patients to participate in their care.11 Pharmacological: Antipsychotic drugs are the primary therapy for all forms of delirium, in particular hyperactive and mixed delirium.12 Table 1 reviews 404 Cancer Control Table 1. — Common Drugs for Pharmacological Management of Delirium Drug Haloperidol Dosage Adverse Events Comment 0.5 mg orally Acute 2–3 times a day extrapyramidal events (eg, torticollis, oculogyric crisis, tongue 1–2 mg intraand laryngeal venous/subcuspasm) taneous every 30 min to 1 h until agitation resolved Mild to moderately agitated elderly patients Risperidone 1 mg orally each day or every other day QT interval and cardiac arrhythmia Potent dopamine blocker Olanzapine 2.5–11 mg daily QT interval and cardiac arrhythmia Very anticholinergic Quetiapine 25–75 mg orally every day QT interval and cardiac arrhythmia Lorazepam 0.5–1 mg intravenous/ subcutaneous Severe agitation in patients aged < 60 y Do not use alone Useful for severe agitation in conjunction with haloperidol Information from reference 13. common agents used for delirium.13 Haloperidol remains the gold standard despite a paucity of clinical trials.14 Severity of delirium and patient age are both important considerations for dosing. In elderly patients (aged ≥ 60 years) and in patients with mild to moderate agitation, Canadian guidelines suggest that haloperidol be started at low doses (ie, 0.5 mg orally 2–3 times a day) and then be titrated to obtain an effect.15 Severe cases and younger patients may require more haloperidol, and, in severely agitated patients, rapid dosing is required. In this setting, parenteral doses are recommended and haloperidol doses such as 1 to 2 mg in young patients and 0.25 to 0.5 mg in elderly patients, repeated every 1 to 2 hours until the agitation resolves; protocols for rapid titration are available.16 Haloperidol is the least sedating of the antipsychotic class and can be intravenously or orally given.17 Atypical antipsychotics may also be useful because they are less likely to cause extrapyramidal symptoms and have less potential for effects on cardiac conduction; however, these antipsychotics cannot be given via the parenteral route.18 Commonly used atypical antipsychotics include olanzapine, quetiapine, and risperidone. These types of antipsychotics are also appropriate for patients with underlying Parkinson disease. Benzodiazepines are not indicated for the management of delirium and are best used in the setting of deOctober 2015, Vol. 22, No. 4 lirium associated with alcohol withdrawal.19 Benzodiazepines can be added to haloperidol when agitation does not respond to haloperidol alone.20 Chronic Nausea and Vomiting Unrelated to Chemotherapy Nausea and vomiting affects up to 70% of patients with advanced cancer; however, nausea is more common.21 Chronic nausea is defined as nausea that lasting more than 1 week without an identifiable precipitant, and its causes include underlying cancer and its progressive effects as well as medication use (eg, opioids). Mechanisms The nausea and vomiting reflex is governed by nuclei in the medulla. The nucleus tractus solitarius receives input from multiple sites, and then relays to the dorsal motor nucleus of the vagus to cause vomiting.22 Afferent input comes from the chemoreceptor trigger zone, vagus nerve, cortex, and vestibular pathways. Neurotransmitters populate these areas critical to the emetic reflex and include dopamine, serotonin, histamine, substance P, and acetylcholine.23 Blocking these neurotransmitters forms the basis for antiemetic therapy. Assessment Health care professionals should gather information about duration, frequency of vomiting episodes, and the ability of the patient to keep fluids down — all of which may affect the route of drug administration. Delayed nausea and vomiting due to chemotherapy should be considered. Large-volume emesis suggests gastric or bowel obstruction, whereas polydipsia, polyuria, and cognitive changes suggest metabolic causes. Health care professionals should inquire about the presence of constipation, which can also cause nausea. Mood should be assessed. Physical examination findings may provide clues. Papilledema suggests brain metastasis, whereas orthostatic changes suggest autonomic insufficiency. Numerical rating scales can be used to measure the severity of nausea. Management Recommendations support either antiemetic therapy based on proposed pathophysiology or a sequential trial of antiemetics.24 Response rates near 80% with these approaches.25 Table 2 lists select antiemetics that can be used in patients with advanced illness.21 Nonpharmacological: Operative approaches are considered in cases of mechanical bowel obstruction. However, consideration for surgical interventions should be individualized, with health care professionals weighing the risks and benefits of the procedure.26 Use of acupuncture to treat nausea and vomiting in advanced illness has not been evaluated.27 October 2015, Vol. 22, No. 4 Dyspnea Dyspnea is the uncomfortable awareness of breathing, and it is a frequent symptom in advanced illness with prognostic importance.28 The prevalence of dyspnea is between 20% and 80% in patients with advanced cancer.28 It has been described in various ways, including as air hunger, suffocation, and choking. Health care professionals typically have differing perspectives on dyspnea, and they often under-rate it as a symptom.28 Mechanisms Peripheral receptors involved in breathing play a role in the perception of dyspnea. Excessive input from receptors sensing oxygen and carbon dioxide levels contribute to dyspnea.29 The overactivity of mechanoreceptors and respiratory muscle as well as lung and chest-wall receptors all contribute to the sensation of dyspnea.28,30 These receptors likely also stimulate the cortex, thus influencing cortical perception.31 Assessment Many tools exist to measure breathlessness, but few Table 2. — Antiemetics in Advanced Illness Drug Dosage Route Comment Butyrophenone Haloperidol 0.5–2.0 mg every 4–6 h Intravenous Oral Subcutaneous Useful with associated delirium Intravenous Oral Subcutaneous Prokinetic Substituted Benzamide Metoclopramide 10 mg every 6h Antihistamine For vestibular component Cannabinoids Dronabinol 5 mg/m2 every 6h Oral Very long half-life (≤ 56 h) Nabilone 1–2 mg twice daily Oral 2-h half-life Not detected on urine testing Glucocorticoid Brain metastasis: 4–6 mg every 6h Intravenous Oral Atypical Antipsychotic Olanzapine 2.5–7.5 mg daily Oral Single daily dose May require another antiemetic for breakthrough emesis Information from reference 21. Cancer Control 405 cancer-specific tools exist; however, one such tool is the Lung Cancer Symptom Scale, and a scale, such as the visual analog scale, numerical pain scale, or cancerspecific tool, is recommended.32,33 Management In advanced illness, treatment should focus on comfort and should include assessment of overall patient distress and work of breathing. Reversible causes of dyspnea and their treatment should be in keeping with the individual patient’s goals of care. Oxygen Therapy: Oxygen therapy can benefit patients with chronic obstructive pulmonary disease and hypoxia in domains such as exercise capacity, cognitive function, and survival; however, its benefits are not clear in the oncology setting.34 Oftentimes, patients with cancer are dyspneic but not hypoxemic. Randomized controlled trials have compared oxygen therapy with room air for relief of dyspnea.35,36 The results of 2 studies suggested that those with cancer who were hypoxemic on room air benefited from oxygen, and the results of another study showed that oxygen did not benefit those with cancer experiencing dyspnea who were not hypoxemic.36,37 Opioids: Opioids are the preferred treatment for refractory dyspnea, and patients typically benefit from oral or parenteral opioids.38 Sustained-release formulations are effective.39 All opioids as a class are effective for dyspnea because they decrease the perception of breathlessness. Results from a metaanalysis of 9 small, randomized studies (116 study patients) and 1 randomized crossover trial (48 study outpatients) showed that systemic opioids reduced mean chronic breathlessness by approximately 20% over baseline.40 No evidence suggests that nebulized opioids are effective.41 Benzodiazepines: Benzodiazepines are used for dyspnea and to relieve anxiety.42 Results from a metaanalysis of 7 studies (200 study patients) found that benzodiazepines did not relieve breathlessness and instead increased the risk of drowsiness; however, the studies were heterogeneous and the sample sizes were small.43 One controlled trial found that adding subcutaneous midazolam to an opioid enhanced dyspnea relief in patients with advanced cancer.44 Antidepressants: Current evidence for the use of antidepressants to decrease breathlessness is inconsistent.45 Saline: A randomized, single-blinded trial of 40 people with an exacerbation of chronic obstructive pulmonary disease showed no consistent relief of breathlessness from nebulized, isotonic saline compared with placebo.46 Furosemide: Exploratory studies are conflicting regarding the efficacy of inhaled furosemide on breathlessness.47 Thus, the level of efficacy of furose406 Cancer Control mide in this patient population requires confirmation in larger clinical trials. Cancer Anorexia/Cachexia Cancer anorexia/cachexia is defined as “a multifactorial syndrome defined by ongoing loss of skeletal mass (with or without loss of fat) that cannot be reversed by conventional nutritional support and leads to progressive functional impairment.”48 A cluster of symptoms characterizes the syndrome and includes weakness, early satiety, and anorexia.48 Criteria for diagnosing cachexia in patients with cancer are: weight loss of more than 5% in 6 months (in the absence of starvation), body mass index below 20 kg/m2, and any degree of weight loss of more than 2% or appendicular skeletal muscle index consisting of sarcopenia (men: < 7.26 kg/m2; women: < 5.45 kg/m2).48 It has 3 stages: precachexia, cachexia, and refractory cachexia. The syndrome occurs in 50% of patients with cancer (many various types).49 Associated complications include poor performance status, poor quality of life, decreased survival, and poor response and tolerability to chemotherapy.48-50 Anorexia frequently accompanies the syndrome.51 Patients may manifest decreased muscle mass and decreased strength, and they may experience fluctuations in resting energy expenditure and increased psychosocial distress.52 Both lean body mass and muscle strength, as measured by handgrip strength, are predictive of survival and quality of life.53 Cancer anorexia/cachexia limits therapeutic options.54 Weight loss correlates with treatment toxicity, poor tumor response, and lower chemotherapy response rates.51 Loss of more than 10% of premorbid weight before chemotherapy predicts death and is independent of disease stage, tumor histology, and performance status.55 Anorexia is also a powerful predictor of early death, and this observation persists even after adjusting for several other prognostic parameters.56 Thus, both weight loss and anorexia predict poor prognosis for patients with advanced cancer. Mechanisms The mechanisms leading to the development of cancer anorexia/cachexia are not fully understood. Anorexia/cachexia results from abnormal host responses to cancer, and the syndrome appears to result from the interplay between tumor byproducts and host cytokine response.57,58 In animal studies, targeting cytokines with monoclonal antibodies relieves cachexia.59 The interplay between tumor byproducts and inflammatory mediators creates acute-phase proteins in the liver, and this, in turn, leads to the breakdown of muscle protein, lipolysis of fat, insulin resistance, and an elevated level of triglycerides.51 Inflammatory mediators enter the hypothalamus, October 2015, Vol. 22, No. 4 which is where they mimic anorexigenic neurotransmitters and cause anorexia.60 Assessment Tools measuring cancer anorexia/cachexia include the Edmonton Symptom Assessment System, Bristol Meyers Anorexia/Cachexia Recovery Instrument, Patient Generated Global Assessment Instrument, and the cancer anorexia/cachexia subdomain of the Functional Assessment of Anorexia/Cachexia Therapy questionnaire.61-63 Clinical tools useful for assessment cancer anorexia/cachexia include weight, midarm circumference, and hand grip.61 It is worth noting that mid-arm circumference has prognostic value.64 Optimal methodology to assess muscle mass has not yet been determined. Computed tomography, dual energy x-ray imaging, and bioimpedance can all be used to measure muscle mass.48 Whole-body impedance and electroconductivity are based on the principle that lean tissue conducts electricity better than fat. Management Secondary reasons for weight loss should be addressed or ruled out. Health care professionals should look for nausea, constipation, taste alteration, depression, dyspnea, and deconditioning as possible causes. Other important patient factors contributing to cancer anorexia/ cachexia include dry mouth, difficulty swallowing, any alteration in sense of smell, early satiety, mouth sores, dental issues, and anxiety. Nutritional Support: Nutritional support early on is important and can include dietary advice, nutritional supplements, and consideration of an enteral diet. Dietitians may recommend foods tailored to preference, portions, and an individual patient’s ability to swallow. Pungent foods should be avoided. It is not clear whether nutritional supplements reverse weight loss and decline in quality of life.65 Psychosocial Intervention: Psychosocial interventions are important because patients and their friends, families, and caregivers may be experiencing distress. Information, support, and clinician interactions with the patient and his or her friends, family, and caregiver can help mitigate such distress. Pharmacologic Intervention Megestrol: Megestrol acetate has long been an option for cancer anorexia/cachexia.66 Results of the initial study (and subsequent studies) showed significant improvement over placebo with respect to appetite, nausea, weight gain, and food intake (megestrol acetate 800 mg/day).67 Bruera et al68 conducted a 15-day, blinded, placebo-controlled, crossover trial of megestrol acetate 480 mg/day and found statistically significant improvements in caloric intake, appetite, and weight gain among the study participants. Results October 2015, Vol. 22, No. 4 of a meta-analysis that included 3,500 participants and 25 clinical trials comparing megestrol acetate with steroids, nandrolone, eicosapentanoic acid, and dronabinol suggested consistent improvement in appetite, weight gain, and quality of life.69 Megestrol acetate improves appetite and weight gain in approximately 30% of patients.69 The numbers of patients needed to treat are 8 patients for weight gain and 3 patients for anorexia.69 Megestrol acetate benefits patients with HIV/AIDS and geriatric cachexia.70,71 Adverse events that occur relatively infrequently include adrenal insufficiency, thrombosis, and hyperglycemia.72-74 Rash and menstrual disorders have similar frequency.67,75 Some evidence suggests that the drug improves fat-free body mass.71 Glucocorticoids: Glucocorticoids work by inhibiting markers of inflammation and also increase neuropeptide Y.76 Glucocorticoids, such as dexamethasone (3–6 mg/day), prednisone (15 mg/day), and methylprednisone (32 mg/day), can improve appetite and can cause weight gain.77 Their effects are rapid but are of short duration than megestrol acetate.78 Glucocorticoids can be used for patients in need of a rapid effect but are more appropriately used in patients with a short prognosis. Cannabinoids: Dronabinol is the most-studied cannabinoid, with dosages ranging from 2.5 to 20 mg/day; compared with placebo, dronabinol reduces nausea, stabilizes weight, and increases appetite.79 Cannabinoids probably inhibit inflammatory mediators, and they also may influence leptin by activating the cannabinoid receptor. Toxicities include euphoria, hallucinations, tachycardia, and psychosis.79 A phase 2 study (N = 19) found that 5 mg/day dronabinol improved anorexia in 68% of study patients, but 16% of study patients discontinued the drug due to adverse events.79 With respect to appetite and weight gain, dronabinol is inferior to megestrol acetate and megestrol acetate/dronabinol combinations, and it is not recommended for cancer anorexia/cachexia.80,81 Anamorelin: Anamorelin is a novel, ghrelin, growth-hormone secretagogue taken orally for the treatment of cancer anorexia/cachexia because it improves appetite and muscle mass.82 Ghrelin is an orexigenic hormone that stimulates food intake in a dose-dependent manner in rodents and humans by stimulating growth hormone and neurotransmitters such as neuropeptide Y. 83,84,85 Levels of ghrelin are elevated in fasting and reduced in obesity.86 It also stimulates the hypothalamus, thus causing food intake.87 The results of a phase 3 trial showed increased lean body mass and decreased symptoms of anorexia/cachexia in patients with stage 3/4 non–small-cell lung cancer.83 Nonsteroidal Anti-Inflammatory Drugs: Nonsteroidal anti-inflammatory drugs (NSAIDs), including cyclooxygenase 2 inhibitors, reduce the tumor-assoCancer Control 407 ciated release of acute-phase reactants and cytokines. The results of 2 controlled clinical trials of patients with cancer anorexia/cachexia suggested that NSAIDs were effective for gaining weight and muscle mass in this study population, and this was especially true when NSAIDs were combined with progestogens.88,89 However, larger trials are required to further study their effect in this patient population.88,89 Thalidomide: Thalidomide has multiple immune-modulating, anti-inflammatory, and TNF-α and IL-6–inhibiting properties. The results of 2 controlled clinical trials show that it increased appetite, weight, and feeling of well being in study patients with cancer anorexia/cachexia.90,91 These initial results are promising, but they may need to be confirmed by additional clinical trials.90,91 Melatonin: Melatonin is an endogenous hormone secreted by the pineal gland and is used to treat sleep disorders. It inhibits cytokines and TNF-α. Two controlled clinical trials measuring the efficacy of melatonin in 1,600 study patients with advanced cancer found improvements in asthenia and anorexia compared with placebo.92,93 However, another controlled clinical trial comparing melatonin with placebo in study patients with cancer anorexia/cachexia (N = 48) did not find any differences between the 2 treatment groups.92 Thus, more studies are indicated to determine its effectiveness. Anabolic Steroids: Oxymetholone, oxandrolone, nandrolone, and fluoxymesterone are anabolic steroids studied in cancer anorexia/cachexia. They act to increase muscle mass without changes in appetite or amount of food intake. They have been evaluated for cachexia with a nononcological origin (eg, AIDS, chronic obstructive pulmonary disease, renal impairment).94-96 One controlled clinical trial confirmed that the efficacy of fluoxymesterone in cancer anorexia/cachexia was comparable with that of megestrol acetate. However, its use is not recommended due to its high rate of hepatotoxicity.78 Combination Treatment: Another approach to cancer anorexia/cachexia is using multiple drugs on different pathways that play a role in the disorder. A controlled clinical trial compared 5 treatments (megestrol acetate, eicosapentaenoic acid, L-carnitine, thalidomide, and combination megestrol acetate/eicosapentaenoic acid/L-carnitine/thalidomide) during a 4-month period.93 The results showed that the drug combination was superior to the drugs alone and improved appetite and asthenia, reduced energy expenditure at rest, increased fat-free mass, and reduced IL-6.93 Fatigue Patients with cancer commonly experience fatigue, which impacts the physical, emotional, and cognitive domains.97,98 Fatigue can impair concentration, often manifesting as lack of motivation.61 In addition, patients may perceive fatigue as decreased energy 408 Cancer Control and weakness.61 Use of radiation, chemotherapy, biological therapies, and targeted therapies may cause fatigue.99,100 Moreover, fatigue can persist in cancer survivors and last for many years.101 Mechanisms Factors contributing to fatigue in patients with cancer can include tumor burden, oncology treatment, and the effect of cytokines.61 Cytokine production leads to symptoms of fever, pain, cachexia, and depression. Patient comorbidities can also exacerbate fatigue and may include chronic obstructive pulmonary disease, renal insufficiency, and electrolyte, endocrine, and preexisting mood disorders.61 Assessment Fatigue should be evaluated like any other symptom, with the health care professional detailing its severity, temporal features, any exacerbating and alleviating factors, associated distress, and its impact on the patient’s daily life. Tools for the measurement of fatigue include the Edmonton Symptom Assessment System, the Functional Assessment of Cancer Therapy-Fatigue Subscale, the Brief Fatigue Inventory, and the Memorial Symptom Assessment Scale Short-Form.102 Management Health care professionals can use nonpharmacological and pharmacological approaches to treat cancerrelated fatigue. Identification of anemia, infection, hypogonadism, pain, depression, anxiety, cachexia, dehydration, metabolic abnormalities, other comorbidities, and medication use, as well as autonomic insufficiency and their attempted correction, are also important. Clear relationships exist between hemoglobin levels and symptoms of fatigue and functional and physical well-being.103 Treating anemia does improve energy and quality of life; however, transfusions can benefit patients with cancer approximately 60% of the time.103,104 Erythropoietin and other similar treatments are impractical in patients with advanced cancer because these treatments often take 4 to 8 weeks.104 As the disease progresses, the benefits of transfusions lessen.104 Nonpharmacological: Exercise can be beneficial for cancer-related fatigue because it improves cardiovascular conditioning, helps patients maintain muscle mass, and can improve mood and sleep.105 Exercise can also benefit patients receiving chemotherapy and bone marrow transplants.106 However, it is important for the health care professional to set realistic expectations for patients when recommending exercise. Patients with cancer may experience metabolic changes, such as an excess lactate level, and morphological muscle changes, such as myofibrillar and sarcomeric abnormalities, even in tumor-free muscles.107-109 DeconditionOctober 2015, Vol. 22, No. 4 ing complicates exercise goals, and use of steroids can cause physical weakness. Thus, an ideal exercise program appropriate for all degrees of performance status among patients with advanced illness remains to be determined. Pharmacological: Management options for cancer-related fatigue include corticosteroids and psychostimulants. Glucocorticoids have been shown to improve fatigue in advanced illness, although their benefits are short term.110 Steroids are best reserved for patients with a short prognosis. Doses equivalent to dexamethasone 8 mg daily are appropriate.111 A metaanalysis of psychostimulants for cancer-related fatigue suggested a benefit for this patient population.112 Psychostimulants such as methylphenidate and modafinil can also be used.113 Conclusions Symptom management in patients with advanced cancer is more effective when health care professionals use a systematic approach to assessment. Expert management requires understanding of the pathophysiology of common symptoms in advanced cancer, use of symptom assessment tools to measure symptom intensity, and the application of proven symptom control interventions. References 1. Bush SH, Bruera E. The assessment and management of delirium in cancer patients. Oncologist. 2009;14(10):1039-1049. 2. Meagher DJ, Leonard M, Donnelly S, et al. A comparison of neuropsychiatric and cognitive profiles in delirium, dementia, comorbid deliriumdementia and cognitively intact controls. J Neurol Neurosurg Psychiatry. 2010;81(8):876-881. 3. Meagher D, Moran M, Raju B et al. A new data-based motor subtype schema for delirium. J Neurol Neurosurg Psychiatry. 2008;20(2):185-193. 4. Stiefel F, Holland J. Delirium in cancer patients. Int Psychogeriatrics. 1991;3(02):333-336. 5. Morita T, Hirai K, Sakaguchi Y, et al. Family-perceived distress from delirium-related symptoms of terminally ill cancer patients. Psychosomatics. 2004;45(2):107-113. 6. Morita T, Tei Y, Inoue S. Impaired communication capacity and agitated delirium in the final week of terminally ill cancer patients: prevalence and identification of research focus. J Pain Symptom Manage. 2003;26(3):827-834. 7. de Stoutz ND, Tapper M, Fainsinger RL. Reversible delirium in terminally ill patients. J Pain Symptom Manage. 1995;10(3):249-253. 8. van der Mast RC. Pathophysiology of delirium. J Geriatr Psychiatry Neurol. 1998;11(3):138-145, 157-158. 9. de Rooij SE, van Munster BC, Korevaar JC, et al. 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Photo courtesy of Lisa Scholder. Solid Stance. 16" × 24". Pharmacological Management of Cancer-Related Pain Eric E. Prommer, MD Background: Pain occurs in 50% of patients with cancer at the time of diagnosis, and nearly 80% of patients with advanced stage cancer have moderate to severe pain. Assessment of pain requires the health care professional to measure pain intensity, delineate opioid responsiveness, and clarify the impact of pain on a patient’s psychological, social, spiritual, and existential domains. To this end, the World Health Organization (WHO) has developed a 3-step pain ladder to help the health care professional effectively manage pain, classifying pain intensity according to severity and recommending analgesic agents based on their strength. Methods: Health care professionals should follow the WHO guidelines to manage cancer-related pain in their patients. With regard to opioids, dosing, equianalgesic conversions, the management of adverse events, and the identification of new agents are discussed. Integrating adjuvant analgesics and interventional pain techniques into the management of cancer-related pain is also discussed. Results: The WHO analgesic ladder is an effective tool for managing cancer-related pain. Successful pain management in patients with cancer relies upon the health care professional to pay attention to detail, especially during the introduction of new drugs and in identifying potential adverse events. Health care professionals must assess opioid responsiveness to determine whether adjuvant analgesics should also play a role in a patient’s treatment plan. Conclusion: Adherence to the WHO pain ladder and understanding proper use of interventional pain techniques complement the pharmacological management of cancer-related pain. Introduction Pain occurs in 50% of patients with cancer at the time of diagnosis, and approximately 80% of patients with advanced stage cancer have moderate to severe pain.1 Pain From the Division of Hematology/Oncology, Veterans Integrated Palliative Care Program, Veterans Integrated Palliative Care, David Geffen School of Medicine, University of California, Los Angeles, California. Submitted July 29, 2015; accepted September 17, 2015. Address correspondence to Eric Prommer, MD, David Geffen School of Medicine, University of California, 11301 Wilshire Boulevard, Building 500, Room 2064A, Mail Code 10P, Los Angeles, CA 90073. E-mail: Eric.prommer@va.gov No significant relationship exists between the author and the companies/organizations whose products or services may be referenced in this article. 412 Cancer Control can impact quality of life, limit function, and affect mood. Untreated pain may sometimes lead to requests for physician-assisted suicide or unnecessary visits to the emergency department and hospital admissions.2,3 Opioids are the cornerstone of treatment for moderate to severe pain associated with cancer because they decrease pain and improve function.4 Strong opioids are the initial choice for moderate to severe pain associated with cancer, and the World Health Organization (WHO) recommends a pain ladder, which is a step-by-step approach for the management of chronic pain based on pain intensity.5,6 Epidemiology Pain occurs at diagnosis in 20% to 50% of patients with cancer.7 Cancer-related pain may be the result of the October 2015, Vol. 22, No. 4 cancer itself, oncology treatment, and coexisting nonmalignant pain.8 Cancer types determine pain prevalence; for example, patients with head and neck cancer have the highest prevalence of cancer pain.9 Age has also been show to affect cancer pain; younger patients experience more pain and more pain flares than older patients.10 In addition, elderly patients receive less opioids than their younger counterparts.11 Patients with cancer most commonly experience pain in the back — prompting health care professionals to exclude spinal cord metastasis — as well as in the abdomen, shoulders, and hips.12 World Health Organization Pain Ladder WHO guidelines form the basis of cancer pain management, recommending a step-by-step approach to managing cancer pain based on pain intensity.2 The pain ladder starts with nonopioid analgesics, such as acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs), for mild pain, then adds a so-called weak opioid if pain persists or increases, and then replaces the weak opioid with a step 3 opioid for severe pain. Morphine is recommended as a first-line opioid to relieve cancer-related pain; however, the evidence level for morphine as a first-line opioid is not particularly strong.3,6 Other step 3 options for moderate to severe pain include methadone, oxycodone, fentanyl, and hydromorphone. Successfully using the WHO pain ladder can help manage pain/provide effective analgesia in 90% of patients in certain settings, although results from randomized control trials show success rates of 70% to 80%.13-15 Step 1 Step 1 analgesics include acetaminophen and NSAIDs, which are both analgesic and anti-inflammatory; acetaminophen is only analgesic.16,17 Dosing of acetaminophen and NSAIDs is limited by a ceiling effect, meaning that further dose escalation will not improve analgesia. Acetaminophen dosing is limited by concerns of hepatic toxicity at a total dose of more than 4 g/day.18 Acetaminophen has several postulated mechanisms of action, including central inhibition of the cyclooxygenase system, nitric oxide synthetase, the endocannabinoid system, and the descending serotonin pathways.17 NSAIDs inhibit the enzyme cyclooxygenase, which produces inflammatory prostaglandins that cause sustained nociceptive responses by lowering pain thresholds in nociceptive, neuropathic, and possibly visceral pain through a process called peripheral sensitization.19 One major action of NSAIDs is the prevention of peripheral sensitization.20 When considering the use of NSAIDs, choices should be based on experience and the toxicity profile, which depends on the cyclooxygenase 1:2 ratio. There is no ideal NSAID. NSAIDs are orally administered, with the exception of October 2015, Vol. 22, No. 4 ketorolac. Thus, loss of the oral route with advanced illness eliminates NSAIDs from consideration for analgesia in patients at the end of life. At the end of life, NSAIDs are typically replaced by stronger analgesics in the setting of moderate to severe pain. It may be important to continue NSAIDs as long as possible, because clinical trials show additive analgesia when combined with opioids as well as an opioid-sparing effect.21 Ketorolac, diclofenac, and ibuprofen are parenteral NSAIDs, and ketorolac is useful for the treatment of cancer pain syndromes not uniformly responsive to opioid therapy.22 Ketorolac use in the advanced patient with cancer is not recommended for more than 1 week.23 Acetaminophen has not been shown to work synergistically with opioids but has not been shown to be opioid-sparing with opioid doses of more than 200 mg of morphine equivalents.22 NSAIDs are useful for pain originating in tissues such as connective tissue, joints, serous membranes, and the periosteum; in addition, visceral pain may also respond to NSAIDs.24 Step 2 Step 2 on the WHO pain ladder is for mild to moderate cancer pain and includes recommendations for acetaminophen products containing hydrocodone, oxycodone, codeine, and tramadol, as well as propoxyphene and dihydrocodeine (not available in the United States).2 Propoxyphene is not recommended for use in cancer pain.25 Hydrocodone: Hydrocodone is structurally similar to morphine, differing only in having a single bond at carbons 7 and 8 and a keto (=O) group at 6-carbon. Hydrocodone is metabolized by both cytochrome P450–dependent oxidative metabolism and glucuronidation. CYP3A4 and CYP2D6 play a role in the generation of hydrocodone metabolites: norhydrocodone and hydromorphone, respectively.26 Polymorphisms of CYP2D6 potentially affect hydrocodone metabolism and therapeutic efficacy.27 Hydrocodone has equivalent potency as morphine on a milligram-for-milligram basis.27 Adverse events of hydrocodone are similar to other opioids. Rodriguez et al28 evaluated 118 study patients with chronic cancer pain and compared hydrocodone/ acetaminophen with tramadol in a double-blind, randomized controlled trial. A total of 62 study patients received hydrocodone/acetaminophen and 56 received tramadol.28 Hydrocodone/acetaminophen decreased pain in 57% of participants at a starting dose of 25 mg/2500 mg/day (5 doses per day).28 Analgesic responses increased by 15% with dose doubling.28 Pain did not respond to hydrocodone/acetaminophen administration in 29% of study patients.28 Another multicenter, double-blind, randomized, parallel group study compared codeine/acetaminoCancer Control 413 phen phosphate with hydrocodone/acetaminophen for moderate to severe pain.29 Study patients had chronic moderate to severe cancer pain (> 3 on a 10-cm visual analog scale and > 1 on a 4-point verbal intensity scale).29 A total of 88% of study patients had moderate pain and 12% had severe pain; 121 participants received either 1 tablet of codeine/acetaminophen 30/500 mg or hydrocodone/acetaminophen 5/500 mg orally every 4 hours (total daily doses, 150/2500 and 25/2500 mg, respectively) for 23 days.29 Dose escalation occurred after 1 week if participants experienced severe pain.29 The primary end point was the percentage of study patients achieving a decrease in their pain score by 1 point on a 5-point verbal intensity scale.29 The secondary end point was the percentage of study patients whose pain decreased by at least 3 cm on the 10-point scale.29 Of the 121 participants, 59 received codeine/acetaminophen and 62 received hydrocodone/acetaminophen.29 Of the total number of cases, 59 had ages ranging from 60 to 89 years.29 A total of 58% of patients in the codeine/acetaminophen arm of the study experienced pain relief, and an additional 8% achieved pain relief with a doubling of the dose.29 Approximately one-third had unresponsive pain.29 In the hydrocodone/acetaminophen arm of the study, 56% experienced pain relief with a starting dose of 25/2500 mg/day.29 A total of 15% more achieved pain relief doubling of the initial dose, and one-third of patients did not respond to hydrocodone/acetaminophen.29 Tramadol: Tramadol is a synthetic opioid from the aminocyclohexanol group. Tramadol has opioid-agonist properties and prevents the uptake of norepinephrine and serotonin, making it useful for neuropathic pain.30 Tramadol possesses low affinity for opioid receptors, with an affinity to μ receptors 10 times weaker than codeine, 60 times weaker than dextropropoxyphene, and 6,000 times weaker than morphine.31 Tramadol requires conversion to an active metabolite by CYP2D6. This metabolite has affinity for opioid receptors, but less so than step 3 opioids.31 Patients who are poor metabolizers of CYP2D6 may experience poor analgesia.32 Adverse events of tramadol include constipation, dizziness, nausea, sedation, dry mouth, and vomiting.33 Rodriguez et al28 evaluated 118 participants with chronic cancer pain and compared hydrocodone/acetaminophen and tramadol in a double-blind, randomized controlled trial. In addition, Wilder-Smith et al34 compared tramadol with morphine in a randomized, crossover, double-blind study for severe cancer pain (N = 20). Initially, participants received either tramadol 50 mg or morphine 16 mg every 4 hours, with dose titration to achieve pain control.34 After 4 days, pain intensities did not differ between the groups, although adverse events appeared to differ, with less-intense 414 Cancer Control nausea and constipation noted in the tramadol group.34 The authors estimated equianalgesic doses of morphine and tramadol and found a ratio of morphine to tramadol of 1:4.34 Tawfik et al35 compared oral tramadol with sustained release morphine for cancer pain in 64 participants with severe cancer pain in a randomized, double-blind study. Tramadol worked best in participants with lesser pain intensity, and morphine worked more effectively and was preferred for participants experiencing severe pain intensity.35 Good analgesia was achieved in 2 weeks of treatment in 88% of study patients receiving tramadol and 100% of study patients receiving sustained-release morphine. Participants receiving tramadol experienced fatigue (15%), nausea (8%), and sweating (8%).35 In those receiving morphine, adverse events included constipation (35%), rash (14%), and drowsiness (14%).35 Bono and Cuffari36 compared tramadol with buprenorphine in a randomized, crossover trial in study patients with cancer pain. All 60 study patients received either drug for 1 week and then, after a 24-hour wash-out period, were switched to the other drug.36 The tramadol dose was 300 mg/day and the buprenorphine dose was 0.2 mg 3 times a day.36 Tramadol was associated with better analgesia (P < .05) and was associated with higher acceptance among study patients.36 Tramadol was better tolerated than buprenorphine and caused less frequent and milder adverse events, and more study drug withdrawals occurred in the buprenorphine arm.36 Tapentadol: Tapentadol is structurally related to tramadol.37 Opioid receptor–binding studies show that tapentadol is a strong opioid with high-affinity binding to µ, δ, and κ opioid receptors. In human µ opioid receptor 35S GTPγS–binding assays, tapentadol shows agonistic activity, with an efficacy of 88% relative to morphine; tapentadol provides potent inhibition of norepinephrine uptake and its bioavailability is lower than tramadol.31 Tmax is achieved in 1.25 to 1.5 hours, the half-life is 24 hours, and the plasma protein binding is 20%.38 Tapentadol metabolism is mainly by glucuronidation, with some contribution from CYP enzymes, especially CYP2D6.39 Tapentadol is not an inducer of CYP3A4.39 Tapentadol has no active metabolites. There is chiefly renal excretion. Tapentadol causes adverse events such as nausea, dizziness, vomiting, headache, and somnolence.40 The manufacturer recommends against using tapentadol in severe hepatic or renal failure, and dosing above 600 mg/day should be avoided.41 Equianalgesic dosing studies are unavailable but information from its use in non–cancer-related pain studies suggest morphine 60 mg is equivalent to tapentadol 100 to 200 mg.42 The current dosing recommendations is 50, 75, or 100 mg every 4 to 6 hours.40 Tapentadol does October 2015, Vol. 22, No. 4 not affect the QTc interval.43 Prolonged-release tapentadol (100–250 mg twice daily) is effective compared with placebo for managing moderate to severe, chronic, malignant tumor-related pain. Codeine: Codeine is a prodrug whose analgesia is mediated through the µ receptor by its metabolite, morphine. A total of 10% of codeine is broken down to morphine by CYP2D6, an enzyme lacking in 5% to 10% of white populations.44 Codeine use is not recommended in the setting of renal failure.45 One placebo-controlled study has evaluated codeine for cancer pain involving a sustained-release formulation.46 Thirty study patients with chronic cancer pain completed the study and received either sustained-release codeine every 12 hours or placebo in a doubleblind study.46 Crossover occurred after 7 days.46 Pain intensity was measured using a visual analog scale as well as a 5-point categorical scale. Rescue analgesia (acetaminophen/codeine 300 mg/30 mg every 4 hours as needed) was recorded. The median doses of controlled-release codeine doses were 277 ± 77 mg (range, 200–400 mg). Pain intensity scores on a visual analog scale, categorical pain intensity scores when assessed by day of treatment and by time of day, and need for breakthrough pain were significantly lower in the codeine arm (P < .0001).53 Step 3 The WHO pain ladder recommends the use of step 3 opioids as first-line therapy for moderate to severe pain (morphine, oxycodone, hydromorphone, fentanyl, levorphanol, methadone).47 Step 3 opioids differ from those in step 2 medications in terms of potency and dosing. Although many step 2 medications often have a ceiling dose due to fixed formulations with acetaminophen, step 3 opioids do not have this ceiling. Dosing can increase to achieve adequate analgesia as long as adverse events are tolerated. Step 3 opioids interact with opioid receptors found throughout the central nervous system and peripheral tissues, resulting in analgesic effects, as well potential adverse events, including sedation, respiratory depression, and dependence. Opioid receptors exist throughout the intestinal tract and, when activated, slow bowel motility.48 Varying degrees of activation and affinity for each receptor subtype may account for the differences in efficacy and activity between opioids. In addition, interindividual variation is significant in analgesic response and toxicities based on genetic disparities.49 However, a reliable method to predict an individual patient’s response does not exist and a paucity of evidence suggests superiority of one opioid over another in terms of efficacy or tolerability. Morphine: WHO considers morphine the drug of choice for moderate to severe cancer pain.50 The liver is the principal site of morphine glucuronidation.51 There October 2015, Vol. 22, No. 4 is a minor contribution (30%) to glucuronidation from the kidneys.52 First-pass metabolism of oral morphine determines its systemic bioavailability. Three major metabolites are produced: normorphine, morphine3-glucuronide, and morphine-6-glucuronide. The metabolites are principally eliminated by the kidney and accumulate in renal failure.53 The elimination half-life of morphine is approximately 2 hours and is independent of route of administration or formulation.54 Morphine administered by sublingual and buccal routes has a delayed onset of action compared with oral morphine (smaller peak plasma levels, lower bioavailability, and larger interpatient variability).54 Intrathecal morphine is 100 times as potent as its oral form, and epidural morphine is 10 times as potent (0.5 mg intrathecally equals 5 mg epidurally).54 Morphine dosing is minimally affected by hepatic failure but is greatly affected by renal failure. There is a linear relationship between creatinine clearance and renal clearance of morphine, morphine-3-glucuronide, and morphine6-glucuronide.55 Kidney failure impairs glucuronide excretion more than morphine excretion, increasing the duration of action of morphine-6-glucuronide and morphine-3-glucuronide, thus leading to adverse events.56 Glucuronidation is largely unaffected by cirrhosis. Morphine doses must be carefully titrated or avoided when creatinine clearance is less than 30 mL/ minute.54 Morphine continues to be considered the standard medication for the treatment of cancer pain partly due to familiarity with the product as well as cost effectiveness. However, it may not always be the ideal product due to issues associated with its metabolism and adverse-event profile.11 Almost all randomized controlled comparisons of potent opioids have shown equivalence (ie, noninferiority) to morphine.6,11,57-59 Methadone: Methadone has features that make it unique: It works at 3 levels to provide analgesia. It is a potent opioid with strong interactions with the µ-opioid receptor, and it is an N-methyl-D-aspartate (NMDA) receptor antagonist, a receptor that is activated in chronic pain states and, when blocked, can enhance analgesia and reverse opioid tolerance. Methadone also works on neurotransmitters, such as norepinephrine and serotonin, which play a role in descending pain modulation.59,60 Methadone is a second-line analgesic for pain that is poorly responsive to other opioids.59 It shows promise as a first-line analgesic for cancer pain, neuropathic pain, and as a breakthrough agent. Methadone is available in oral, sublingual, and intravenous formulations. Methadone has different pharmacokinetics from other opioids. Methadone has a long half-life that varies between 60 and 120 hours.59 High-dose intravenous methadone is associated with QT prolongation and torsades de pointes.61 In fact, a retrospective study found Cancer Control 415 that oral methadone can cause QT prolongation in 16% of patients.62 Dosing of methadone is complicated. Methadone shows an inverse relationship of its starting dose to the total morphine equivalent daily dose (MEDD). As the MEDD increases, the equianalgesic dose of methadone progressively decreases. Clinical trials comparing methadone to morphine have not shown superiority of methadone; in fact, 3 studies have compared morphine and methadone as first-line therapy for cancer-related pain.6,63,64 Ventafridda et al63 compared methadone with morphine for moderate to severe cancer pain in 54 study patients who had previously been taking step 2 opioids. Patients received either morphine or methadone by mouth for 14 days.63 Both therapies provided clear reductions in pain intensity, there was less stability in analgesia in the morphine arm, and study patients receiving morphine had a higher incidence of dry mouth.63 Otherwise, no other differences in toxicities or the ability to achieve pain relief were seen.63 Mercadante et al64 conducted a prospective randomized study in 40 study patients with advanced cancer who required strong opioids for their pain management and receiving home hospice care. Study patients were treated with sustained-release morphine or methadone in doses titrated to pain relief and administered 2 or 3 times daily according to clinical need.64 Results suggested that methadone more quickly achieved analgesia and methadone analgesia was more stable than that achieved with morphine.64 Bruera et al6 compared the effectiveness and adverse events of methadone and morphine as first-line treatment with opioids for cancer pain. In this multicenter, international study, 103 participants with pain requiring strong opioids were randomly assigned to receive either methadone or morphine for 4 weeks. Participants with 20% or more reductions in pain scores were equal in both groups. Those in both arms reported satisfaction with their therapies. The methadone arm had a higher number of dropouts and required fewer dose adjustments to achieve analgesia than those in the morphine arm.6,11 Hydromorphone: Hydromorphone is similar in structure to morphine and is available as parenteral and oral products. It is the best opioid for subcutaneous administration.65 The oral formulation is available in an immediate-release formulation, and a single, daily dose, extended-release formulation has been shown to be effective in patients with cancer.66 Administered orally, hydromorphone has a bioavailability of 50% and its plasma elimination half-life is 2.5 hours.67 Metabolism in the liver produces hydromorphone-3-glucuronide, which has no analgesic properties but can cause neurotoxicity.68 Hydromorphone is effective in treating pain in patients with renal impairment. Hydromorphone metabolites accumulate in patients receiving chronic infusions.69 A double-blind, randomized com416 Cancer Control parison of sustained-release hydromorphone with sustained-release morphine showed equivalence in pain relief.70 Systematic reviews involving 11 studies and 645 study patients show that hydromorphone equals morphine in analgesic effect.71 Oxycodone: Oxycodone is available as immediate-release and sustained-release formulations. (Intravenous formulations are available in Europe.) The immediate-release formulation has a half-life of approximately 2 to 4 hours and a bioavailability of 50% to 60%.72 The primary difference between oxycodone and morphine is its bioavailability: its half-life is longer than normal in renal failure and liver failure.45,73 Several trials comparing oxycodone with morphine show equal efficacy.72,74 Minor differences in adverse events have been described.74 Hallucinations and nausea are less common with oxycodone treatment.75 However, because of its cost and lack of versatility, morphine remains the preferred analgesic.76 Bruera et al74 demonstrated that oxycodone is 1.5 times as potent as morphine when comparing analgesic potency. Oxymorphone: Oxymorphone is a semisynthetic μ-opioid agonist 1.2 times as potent as morphine.77 Until recently, oxymorphone was available as parenteral injection and in suppository form; however, immediate-release and long-acting oral formulations were developed that make oxymorphone another option for treating moderate to severe pain. Trials in malignant and nonmalignant pain confirm its potential as another step 3 option.77 Oxymorphone is more lipid soluble than morphine. The oral bioavailability of oxymorphone is approximately 10%, which is the lowest of the oral step 3 opioids.77 In healthy volunteers, the half-life ranges from 7.2 to 9.4 hours. The half-life of immediate-release oxymorphone is longer than that of morphine, hydromorphone, and oxycodone.78 Immediaterelease oxymorphone tablets may be given at 6-hour intervals, whereas the extended-release formula is dosed twice daily. Steady-state conditions are achieved after 3 to 4 days. Oxymorphone is subject to hepatic first-pass effects and is excreted by the kidneys. Oxymorphone accumulates in renal failure. Oxymorphone has a prolonged half-life in renal failure.79 In the setting of hepatic insufficiency, increasing the dosing interval is recomended.80 Sloan et al81 conducted a pilot study comparing extended-release oxymorphone and controlled-release oxycodone in 86 study patients with moderate to severe cancer pain. The tolerability and safety profiles (eg, nausea, drowsiness, somnolence) were similar between the 2 drugs, and no significant differences in daily pain intensity scores were seen between extended-release oxymorphone and oxycodone.81 Fentanyl: Fentanyl, a lipid-soluble, synthetic opioid, is available as parenteral, transdermal, and transmucosal products. Its lipophilic properties allow it to October 2015, Vol. 22, No. 4 cross both the skin and oral mucosa.82 The transdermal formulation delivers fentanyl from the reservoir into the stratum corneum where it then slowly diffuses into the blood. Another formulation on the market is a matrix-delivery system in which fentanyl is dissolved in a polyacrylate adhesive. This formulation can be cut.83 Both the reservoir and matrix-based patches have similar kinetics and clinical effectiveness.83 Fentanyl is metabolized to norfentanyl under the influence of CYP3A4.84 The concomitant use of fentanyl with potent CYP3A4 inhibitors (eg, ritonavir, ketoconazole) may affect its metabolism. Fentanyl is safe to use in patients with renal failure.60 The elimination half-life of transdermal fentanyl is approximately 12 hours. Conversions to fentanyl are made by calculating the MEDD and the using the ratio of 2 mg:1 µg to reach the starting fentanyl dose.82 Most experts do not recommend using transdermal fentanyl for acute titration.85,86 Compared with morphine, constipation is less frequent with fentanyl.87 Comparisons between morphine and transdermal fentanyl have shown equal analgesic efficacy.88 When compared with morphine, daytime drowsiness and interference with daytime activity occur at lower rates.88 The oral transmucosal administration of fentanyl has been extensively explored. In 1 study, 25% of the delivered drug was transmucosally absorbed, with another 25% delivered through the gastrointestinal tract.82 Randomized controlled trials of oral transmucosal fentanyl citrate show increased analgesic efficacy and patient preference over placebo and morphine.89,90 Administration of fentanyl is being explored through other routes (eg, intranasal).91 Rapid intravenous administration of fentanyl in the emergency department can result in rapid improvement in pain control.92 Buprenorphine: Buprenorphine is emerging as another option for cancer pain. Well-known as a strong analgesic, the development of a transdermal formulation makes it a possible option for cancer pain.93,94 Buprenorphine is also available in intravenous and sublingual formulations, with the sublingual formulation having a bioavailability of 50% to 65% and a half-life of more than 24 hours.95 After application of the transdermal formulation, plasma concentrations steadily increase. The larger-dose transdermal formulations achieve the minimum effective therapeutic dose sooner. Open-label, randomized, parallel-group, multipledose pharmacokinetic studies show that the minimum effective concentrations are reached after 31, 14, and 13 hours, respectively, with the 35, 52.5, and 70 mg/hour patches (not available in the United States).96 Patches reach steady state after the third consecutive application.97 Bioavailability of the transdermal formulation is 60% compared with the intravenous route.98 Effective plasma levels occur within 12 to 24 hours and last for October 2015, Vol. 22, No. 4 72 hours. It takes 60 hours to reach Cmax. After patch removal, concentrations decrease to one-half in 12 hours, then more gradually decline.96 Metabolism by CYP3A4 and CYP2C8 converts buprenorphine to an active metabolite, norbuprenorphine, which is a weaker but full-opioid agonist. Buprenorphine and its metabolite later experience glucuronidation.99 Liver disease affects buprenorphine metabolism. With involvement of both cytochrome oxidase system and glucuronidation in metabolism, severe liver disease potentially inhibits formation of norbuprenorphine through effects on the cytochrome oxidase system. Liver disease does not affect glucuronidation as much. Buprenorphine is safe to use in the presence of mild to moderate liver failure as well as in the setting of renal insufficiency and dialysis.94,100 Buprenorphine produces adverse events similar to other step 3 opioids and include constipation, urinary retention, sedation, and cognitive dysfunction. Buprenorphine causes less nausea than transdermal fentanyl.101 Three phase 3, placebo-controlled studies of mixed study populations with cancer evaluated transdermal buprenorphine for cancer pain.102-104 In these studies, buprenorphine acted as an opioid agonist. There was no dose ceiling or opioid antagonist activity. Levorphanol: Levorphanol is a potent opioid considered to be similar to methadone.105 Morphologically similar to morphine, levorphanol has strong affinity for μ, δ, and κ opioid receptors.106 Levorphanol is a noncompetitive NMDA receptor antagonist and blocks NMDA with the same potency as ketamine.107 Levorphanol can be orally, intravenously, subcutaneously, and intramuscularly administered.108 Levorphanol has poor absorption via the sublingual route compared with other opioids such as morphine sulfate (18%), buprenorphine (55%), fentanyl (51%), and methadone (34%).109 The pharmacokinetics of levorphanol are similar to methadone with a duration of analgesia ranging from 6 to 15 hours and a half-life as long as 30 hours.110 First-pass metabolism produces a 3-glucuronide metabolite, which may have neurotoxicity.110 Metabolites of levorphanol are renally excreted. The high volume of distribution and increased protein binding suggest that levorphanol should not be dialyzable. In the setting of renal disease, the dosing interval should be increased. This differs from methadone. The predominant mode of metabolism is hepatic. In the setting of hepatic insufficiency, it is advisable to consider an increased dosing interval.108 Experience and clinical trial results suggest that the type and incidence of adverse events are similar to those seen with strong opioids.108 Levorphanol has been studied as a treatment for chronic neuropathic pain and has been shown to be effective.111 Cancer Control 417 Interventional Pain Modalities Clinicians consider “step 4” of the WHO pain ladder when there is an inadequate response to step 3 agents, adjuvants, or both.112 Treatment options include use of nerve blocks, as well as spinal administration of local anesthetics, opioids, and other adjuvants. Abdominal pain may be controlled by a blockade of the celiac plexus, which, if successful, can block nociceptive input from many structures in the upper abdomen, in particular the pancreas.113 Use of the superior hypogastric ganglion block for the treatment of malignant pelvic pain was first described by Plancarte et al.114 Opioids Receptor Interactions Opioids interact with opioid receptors to produce analgesia (as well as adverse events).115,116 Opioids interact with receptors, leading to receptor phosphorylation by G protein-coupled receptor kinases. Arrestin then binds with the activation of distal pathways.117 Opioids intracellularly drive receptors by endocytosis, with the receptors ultimately resurfacing.117 Opioids differ in their G protein coupling and in their propensity to drive receptors into the cell. For example, compared with other strong opioids, morphine is inefficient in its ability to promote receptor internalization.117 Some postulate that noninternalized receptors continue to signal and promote adaptive responses, thus causing cellular tolerance.117 Responsiveness Opioid responsiveness is the “degree of analgesia achieved as the opioid dose is titrated to an endpoint, defined either by intolerable side effects or the occurrence of acceptable analgesia.”118 Pain poorly responsive to opioids exists when intolerable adverse events, inadequate analgesia, or both continue despite opioid escalation. Pharmacodynamic and nonpharmacodynamic factors affect opioid responsiveness. Identifying pain poorly responsive to opioids should lead the health care professional to consider using adjuvant analgesics or opioid switching, changing the route of administration, using NMDA antagonists, or interventional pain techniques.113,119-121 Routes of Administration Opioids are available in many dosage forms, including via the oral, rectal, subcutaneous, intramuscular, intravenous, transdermal, transmucosal, and intraspinal routes of administration. Oral administration is simple, cost effective, and is the preferred route of delivery. Both immediate-release and extended-release preparations are available. Clinicians use the subcutaneous, intravenous, rectal, transdermal, transmucosal, and intraspinal routes when patients cannot take oral medications. Intramus418 Cancer Control cular administration is contraindicated as it does not confer any pharmacokinetic advantages and is painful for patients.122 Subcutaneous delivery is relatively easy, effective, and safe.123 Intravenous routes are useful when pain is severe or pain levels have acutely increased. Transdermal fentanyl preparations are effective for patients unable to take oral medications and have stable pain control. Other short-acting opioids are used to control pain when transdermal fentanyl is used, because levels of fentanyl gradually increase during a 12- to 24-hour period until reaching steady state.124 Transmucosal fentanyl is similar to intravenous administration in its rapid onset, and it can be used for acute breakthrough pain. Historically, dosing of transmucosal fentanyl was not thought to be based on dose proportionality, but this consideration has been challenged.125 Intraspinal administration of opioids can either be epidural or intrathecal. This method is the most invasive technique and requires a specialist for initiation. This delivery confers advantages in patients with significant dose-limiting adverse events as systemic involvement is circumvented. Intraspinal delivery allows the addition of adjuvant medications to opioids that can be directly administered to the spinal cord.126 Dose Titration Clinicians adjust opioid analgesics to balance adequate pain control with their respective adverse events. Dosage requirements change with cancer progression. Most patients with cancer have chronic daily pain, so analgesics should be given on a scheduled basis.121 Breakthrough analgesics are ideally given according to the time it takes to reach C max. The C max depends on the route of administration. Cmax is 1 hour for the oral route, 30 minutes for the subcutaneous route, and 6 minutes for the intravenous route.127,128 Once Cmax is reached, another dose should be given if pain is not adequately controlled. Multiple approaches to opioid initiation and titration exist. The European Association for Palliative Care recommends dose titration with immediaterelease oral morphine every 4 hours, with breakthrough dosing of the same dose given every hour as needed.129 The scheduled dose should then be adjusted to account for the oral MEDD. Several studies have shown acceptable pain control and adverseevent profiles with use of 5 mg every 4 hours in study patients naive to opioids and 10 mg every 4 hours in patients previously using a step 2 drug.129-131 After acceptable pain control occurs, patients can use extended-release preparations as this is convenient and improves compliance.132 Breakthrough dosing is 10% to 20% of the MEDD.133 Opioid titration with sustained-release formulations is slower than titration with immediate-release formulations.129 Titration with intravenous medications October 2015, Vol. 22, No. 4 is effective and tolerated.134 In patients on established opioid regimens, dosing adjustment should be made according to the level of pain. Adult cancer pain guidelines recommend an increase of 25% to 50% in the total MEDD for moderate pain (4–6 out of 10) and 50% to 100% for severe pain (7–10 out of 10).133 Equianalgesic Conversions When converting between opioids, equianalgesic guidelines should be followed, although they may be modified according to clinical judgment with regard to adequacy of a patient’s current pain medication regimen.135 Opioid rotation may be secondary to poor analgesia, excessive adverse events, convenience, or patient preference.136 Incomplete cross tolerance is a phenomenon that has been empirically observed.135 For various reasons, patients may develop less of a response (eg, poor analgesia, adverse events) to a particular opioid over time. Patients may not show these characteristics with a new opioid, despite similar action between opioids, and slight variations in opioid structures may account for this.137 When calculating the dose of the new opioid, new doses should be reduced by 25% to 50% to account for non–cross tolerance.133 This is not done for fentanyl or methadone, and equianalgesic guidelines should not to be used for these calculations. Adverse Events The development of adverse events varies between individuals based on age, comorbidities, stage of illness, and genetic differences.116 Impaired renal function also increases the risk of adverse events due to accumulation of active metabolites.116 The most common adverse events include constipation, nausea, vomiting, and altered cognition.116 Other adverse events may include xerostomia, urinary retention, respiratory depression, myoclonus, pruritus, and hyperalgesia.116 Most adverse events from opioid use subside within days to weeks, except for constipation for which patients do not develop tolerance and is not dose-related. For those symptoms that persist or are present during the initiation of opioid therapy, symptom management is a key element of care. Constipation is prophylactically managed. Opioids inhibit gastrointestinal peristalsis; thus, all patients should receive a stimulant laxative such as senna, which can be combined with a stool softener such as docusate sodium or polyethylene glycol. Dietary recommendations, such as increasing fiber in the diet, are unrealistic in patients with advanced disease because hydration is necessary to facilitate the action of fiber, often something difficult to achieve in ill patients.126 Constipation is exacerbated by metabolic abnormalities, including diabetes, hypercalcemia, hypokalemia, and hypothyroidism, that should be corrected if possible.116 Increased physical activity is often helpful if possible. Use of quaternary opioid antagoOctober 2015, Vol. 22, No. 4 nists may be needed.116 The quaternary agents do not cross the blood–brain barrier and do not reverse the analgesic effects of opioids. Nausea frequently occurs at the start of opioid therapy but seldom persists. Ongoing nausea may occur with advanced disease or as a complication of disease treatments. Opioids can cause nausea through several mechanisms, either through direct stimulation of the chemoreceptor trigger zone, increased sensitivity of the vestibular apparatus, or delayed gastric emptying.116 Management consists of therapies targeting these processes. Dopamine antagonists, such as prochlorperazine or haloperidol, work on the chemoreceptor trigger zone. Antihistamines or anticholinergics can be used in patients who have nausea associated with movement. Metoclopramide is both a dopamine antagonist and promotility agent commonly used for the treatment of nausea in palliative care. Ondansetron, a serotonin receptor antagonist, is also a first-line agent for the management of nausea.116,138 If sedation and altered sensorium are present, then management should include evaluation for other sources such as dehydration, drug interactions, or disease progression. Studies have investigated use of stimulants such as methylphenidate and modafinil with varying results.139,140 If excessive adverse events limit pain control or impair quality of life, opioid rotation is often effective at achieving greater pain control with less adverse events.136 In addition, this method of adverse-event management is preferable in patients for whom polypharmacy is a concern. Based on pharmacodynamic studies, dose-response relationships exist for central nervous system effects, such as sedation, myoclonus, and delirium, and may improve with dose reduction.116 Treating Neuropathic Pain Although adjuvant analgesics are often used in neuropathic pain, health care professionals should consider opioids as another option for neuropathic pain. Opioids are recommended as part of neuropathic pain algorithms.141 Adjuvant Analgesics Adjuvant analgesics are drugs with a primary indication other than pain that have analgesic properties.142 The group includes drugs such as antidepressants, anticonvulsants, corticosteroids, neuroleptics, and other drugs with narrower adjuvant functions. Adjuvant analgesics are particularly useful when evidence of decreased opioid responsiveness is present. Tricyclic Antidepressants and Selective Serotonin Reuptake Inhibitors The tricyclic antidepressants have been studied for use in neuropathic pain syndromes, although study results are conflicting about their analgesic effectiveness.143-145 Cancer Control 419 Use in the elderly may also be problematic due to adverse events, including orthostatic hypotension and sedation.146 Tricyclic antidepressants should also be cautiously used in patients with coronary artery disease or cardiac rhythm disorders, as well as those with a history of narrow anterior eye chambers or glaucoma. The anticholinergic properties of these drugs contribute to delirium in the elderly or anyone at risk for delirium such as patients whose cancer has metastasized to the central nervous system. These drugs should be started at the lowest dose with cautious escalation. Dose escalations are made every 3 to 4 days if analgesic response is suboptimal. Selective serotonin reuptake inhibitors (SSRIs) have a limited role as adjuvants, although paroxetine and citalopram have been evaluated for nonmalignant neuropathic pain.147,148 No studies have been performed on cancer pain. Some SSRIs have unique mechanisms of action that may make them useful for cancer pain; for example, venlafaxine, which inhibits the uptake of serotonin and norepinephrine (important in the regulation of descending pain pathways), is effective for painful neuropathy and neuropathic pain associated with therapy used in breast cancer.148,149 Newer drugs, such as duloxetine, can be used to inhibit the uptake of norepinephrine, which is also effective in neuropathic pain, especially related to chemotherapy.150 Bupropion, a noradrenergic compound, has both analgesic and activating properties and can be effective in patients with depressionand significant neuropathic pain.151 Corticosteroids Corticosteroids can be used for patients with bone pain and to decrease swelling in the brain and spinal cord due to metastatic disease. Nerve root inflammation responds to corticosteroids. Corticosteroids are often considered for painful liver metastasis and obstruction of the ureter, although the evidence base for this use is not strong.152 The most commonly used corticosteroid is dexamethasone, which has low mineralcorticoid properties. Optimal dosing for palliation may be 8 mg as this dose has no more adverse events than placebo.153 In the case of spinal cord compression, recommendations exist for either high-dose (96 mg/day) or low-dose (16 mg/day) dexamethasone.154 The challenge with the higher dose of steroids is the occurrence of adverse events.155 The management of edema associated with brain metastasis can be treated with dexamethasone 4 to 6 mg every 6 hours with a taper during the last phases of palliative radiation therapy. The minimal effective dose for brain metastasis is 8 mg/day.156 Steroids can be useful to counteract the phenomenon of radiation “flare,” which can occur with radiation therapy when radiation is applied to painful bony sites.157 420 Cancer Control Anticonvulsant Drugs Anticonvulsants can be used for managing neuropathic pain.158 The most often used anticonvulsant for neuropathic pain is gabapentin. Gabapentin is effective for cancer-related neuropathic pain.159 Gabapentin can have significant adverse events if it is started at too high a dose or titrated too fast. Dosing begins at 150 mg to 300 mg at bedtime, with escalations every 3 days if pain control is suboptimal. The maximum dose is 3600 mg/day. The chief adverse event is somnolence.160 Gabapentin must be dose adjusted for renal insufficiency. Anoother anticonvulsant that may be useful for cancer pain is phenytoin.161 Agents such as lamotrigine, oxcarbazepine, pregabalin, topiramate and levetiracetam have been used for nonmalignant neuropathic pain and are considerations in the refractory case, but they have not been studied in the cancer pain population. Levetircetam requires further study for cancer-related neuropathy.162 Lamotrigine is not effective in chemotherapy-related neuropathy.163 Oral and Parenteral Local Anesthetics The most common parenteral anesthetic used for symptom management is lidocaine.164 Studies suggest its efficacy in refractory cases of neuropathic pain. One study in patients with cancer with refractory pain showed improved analgesia with a single dose of lidocaine.165 The recommended starting dose is 1 to 5 mg/kg infused for 20 to 30 minutes. In patients who are frail, lower doses may be needed. Lidocaine should be avoided in patients with coronary artery disease. One potential benefit of lidocaine is prolonged pain relief that occurs following its infusion. Lidocaine can be given subcutaneously in the home or hospice setting.166 Mexililetine, an oral cogener of lidocaine, has been used after lidocaine infusions.167 Clinical trial results suggest that mexiletine has a distinct adverse-event profile and may not be tolerated by all patients.168 Transdermal Analgesics Transdermal lidocaine (5% patch) provides another route for local anesthetics. It can be used to treat postherpetic neuralgia, but use in other settings requires further study to clarify its role in cancer-related neuropathy. The patch has minimal systemic absorption, and it can be applied 12 hours per day; evidence suggests that increasing the number of patches and extended dosing periods may be safe.169,170 It may take several weeks to observe a maximal effect. The most frequently reported adverse events are mild to moderate skin redness, rash, and irritation at the patch application site.105 Ketamine Chronic pain is associated with central nervous system October 2015, Vol. 22, No. 4 changes, including activation of the NMDA receptor, and can lead to opioid tolerance and the development of opioid resistance.171 Pharmacological blockade of the NMDA receptor offers a therapeutic approach in the setting of opioid resistance. Ketamine is a useful NMDA antagonist to consider in the management of cancer pain and its use often leads to reduced opioid requirements.172 Given at subanesthetic doses (< 1 mg/ kg), ketamine is an effective analgesic in cancer-related neuropathic pain.172 Multiple routes exist for administration and include the oral, intravenous, subcutaneous, and topical routes. Ketamine is metabolized via CYP3A4. No significant drug interactions have been reported.173 Ketamine is recommended by the WHO for the management of refractory pain.174 The oral bioavailability is 17%, and onset of action of ketamine is 15 to 20 minutes. The half-life of ketamine is 2.5 to 3 hours. Ketamine has protein binding of 20% to 30%.175 Pharmacologically, no major differences exist in the characteristics between the isomers.176 Its intravenous onset of action is within seconds and, subcutaneously, the onset of action is 15 to 20 minutes. The half-life is 2 to 3 hours for both routes.173 The results of 1 trial of subcutaneous ketamine as an add-on option to opioids showed no efficacy in cancer-related nociceptive pain.177 Cannabinoids Formulations of cannabinoids, the cannabinoid extracts, have been studied for cancer-related pain.178 Johnson et al179 evaluated tetrahydrocannabinol (THC)/cannabidiol (CBD) in a 2-week, multicenter, double-blind, randomized, placebo-controlled, parallel-group trial of 177 study patients with cancer whose pain was inadequately controlled despite them being on opioid therapy. The cannabinoid extract contains THC 2.7 mg and CBD 2.5 mg per dose.179 It is formulated in ethanol/propylene glycol with peppermint flavoring and is designed as a pump spray for self-administration and titration via the oromucosal route.180 The study patients received THC/CBD, THC extract, or placebo and continued their previous analgesics.179 The THC/CBD extract arm achieved a statistically significant improvement in pain when compared with placebo (P < .024) as measured on a numerical rating scale, a primary end point of the study.179 The THC extract showed no significant changes from baseline compared with placebo.179 Neuroleptics Second-generation (atypical) agents, such as olanzapine, have been shown to have antinociceptive activity in animal models.181 Clinical evaluation of its analgesic effects has been limited. Khojainova et al182 evaluated the analgesic activity of olanzapine in 8 study patients with severe cancer pain who did not respond to increased opioid dosing and who also received olanOctober 2015, Vol. 22, No. 4 zapine for the treatment of associated anxiety and mild cognitive impairment. Participants did not meet diagnostic criteria for delirium and the cognitive impairment was classified as not otherwise specified.182 Study patients received 2.5 to 7.5 mg of olanzapine daily, and their pain intensity, sedation, and opioid consumption measurements were made before administering olanzapine and 2 days after olanzapine was given.182 Cognitive function was assessed daily.182 All participants experienced reduced pain scores, and the average daily opioid use significantly decreased in all study patients.182 Cognitive impairment and anxiety resolved within 24 hours of initiating olanzapine.182 The authors suggested that olanzapine may have an intrinsic analgesic action, but they also suggested that pain scores and opioid requirements may have resulted from improvement in cognitive function and the known anxiolytic effect of olanzapine.182 Agents Specifically Used for Bone Pain Bone pain is a common problem in the palliative care setting. Radiation therapy can be effective with localized pain. Systemic therapies with NSAIDs, corticosteroids, bisphosphonates, and radiopharmaceuticals can be useful for patients with multifocal lesions. Bisphosphonates: Bisphosphonates are analogues of inorganic pyrophosphate that inhibit osteoclast activity and can be useful in many types of cancer in which bone resorption leads to complications. Bisphosphonates bind to calcium on bone, become ingested by osteoclasts, and then subsequently kill osteoclasts, thus preventing bone resoprtion.183 The end result of decreased osteoclast activity is increased bone stability and reduced pathological fractures. The most potent bisphosphonate is zoledronic acid, which has been shown to reduce pain and the occurrence of skeletal-related events in breast and prostate cancers, multiple myeloma, and a variety of solid tumors, including lung cancer.184-187 Denosumab is useful when renal insufficiency precludes the use of bisphosphonates.188 Radiopharmaceuticals: Radionuclides are agents absorbed in areas of metastatic cancer activity. Strontium-89 and samarium-153 are effective for diffuse bony metastatic disease, such as in the case of prostate cancer.189 Muscle Relaxants Pain originating from connective tissue injury is common in patients with cancer. However, use of muscle relaxants as adjuvant agents has not been evaluated in patients with cancer. Use for Malignant Bowel Obstruction Pain, along with nausea and vomiting, is a common symptom associated with malignant bowel obstruction. Nonsurgical management of malignant bowel obCancer Control 421 struction focuses on the management of pain and other obstructive symptoms, such as distension, nausea, and vomiting. The use of parenteral opioids, antiemetics, and antisecretory agents, such as octreotide, are common methods of pharmacological symptom control. Octreotide has anecdotally been shown to have analgesic properties.190 Combination Use The treatment of neuropathic pain frequently requires several adjuvants. For example, it is not unusual for a patient with severe, cancer-related neuropathic pain to require an opioid or several additional adjuvants. When this occurs, the clinician should monitor the patient for potential drug interactions.191 Conclusions Successful cancer pain management requires close attention to detail, particularly when introducing the drug; in addition, health care professionals must be watchful for the presence of adverse events. 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Analgesic effect of intravenous ketamine in cancer patients on morphine therapy: a randomized, controlled, double-blind, crossover, double-dose study. J Symptom Pain Manage. 2000;20(4):246-252. 121. Bruera E, Kim HN. Cancer pain. JAMA. 2003;290(18):2476-2479. 122. Mercadante S, Radbruch L, Caraceni A, et al. Episodic (breakthrough) pain. Cancer. 2002;94(3):832-839. 123. Radbruch L, Trottenberg P, Elsner F, et al. Systematic review of the role of alternative application routes for opioid treatment for moderate to severe cancer pain: an EPCRC opioid guidelines project. Palliat Med. 2011;25(5):578-596. 124.Muijsers RB, Wagstaff AJ. Transdermal fentanyl. Drugs. 2001; 61(15):2289-2307. 125. Vasisht N, Gever LN, Tagarro I, et al. Formulation selection and pharmacokinetic comparison of fentanyl buccal soluble film with oral transmucosal fentanyl citrate. Clin Drug Invest. 2009;29(10):647-654. 126. Thomas JR, von Gunten CF. Pain in terminally ill patients. CNS Drugs. 2003;17(9):621-631. 127.Collins S, Faura C, Moore RA, et al. Peak plasma concentrations after oral morphine: a systematic review. J Pain Symptom Manage. 1998;16(6):388-402. 128. Stuart-Harris R, Joel SP, McDonald P, et al. The pharmacokinetics of morphine and morphine glucuronide metabolites after subcutaneous bolus injection and subcutaneous infusion of morphine. Br J Clin Pharmacol. 2000;49(3):207-214. 129. Klepstad P, Kaasa S, Skauge M, et al. Pain intensity and side effects during titration of morphine to cancer patients using a fixed schedule dose escalation. Acta Anaesthesiol Scand. 2000;44(6):656-664. 130. De Conno F, Ripamonti C, Fagnoni E, et al. The MERITO study: a multicentre trial of the analgesic effect and tolerability of normal-release oral morphine during ‘titration phase’ in patients with cancer pain. Palliat Med. 2008;22(3):214-221. 131. Gatti A, Reale C, Occhioni R, et al. Standard therapy with opioids in chronic pain management. Clin Drug Invest. 2009;29(1):17-23. 132. McCarberg BH, Barkin RL. Long-acting opioids for chronic pain: pharmacotherapeutic opportunities to enhance compliance, quality of life, and analgesia. Am J Ther. 2001;8(3):181-186. 133. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Adult Cancer Pain. v2.2015. http://www.nccn.org/ professionals/physician_gls/pdf/pain.pdf. Accessed October 2, 2015. 424 Cancer Control 134.Radbruch L, Loick G, Schulzeck S, et al. Intravenous titration with morphine for severe cancer pain: report of 28 cases. Clin J Pain. 1999;15(3):173-178. 135. Knotkova H, Fine PG, Portenoy RK. Opioid rotation: the science and the limitations of the equianalgesic dose table. J Pain Symptom Manage. 2009;38(3):426-439. 136. Mercadante S, Ferrera P, Villari P, et al. Frequency, indications, outcomes, and predictive factors of opioid switching in an acute palliative care unit. J Pain Symptom Manage. 2009;37(4):632-641. 137. Pasternak GW. Incomplete cross tolerance and multiple mu opioid peptide receptors. Trends Pharmacol Sci. 2001;22(2):67-70. 138. Mystakidou K, Befon S, Liossi C, et al. Comparison of tropisetron and chlorpromazine combinations in the control of nausea and vomiting in patients with advanced cancer. J Pain Symptom Manage. 1998;15(3):176-184. 139. Peuckmann V, Elsner F, Krumm N, et al. Pharmacological treatments for fatigue associated with palliative care. Cochrane Database Syst Rev. 2010;(11):CD006788. 140. Webster L, Andrews M, Stoddard G. Modafinil treatment of opioidinduced sedation. Pain Med. 2003;4(2):135-140. 141. Dworkin RH, Backonja M, Rowbotham MC, et al. Advances in neuropathic pain: diagnosis, mechanisms, and treatment recommendations. Arch Neurol. 2003;60(11):1524-1534. 142. Lussier D, Huskey AG, Portenoy RK. Adjuvant analgesics in cancer pain management. Oncologist. 2004;9(5):571-591. 143. Tiina T. Amitriptyline effectively relieves neuropathic pain following treatment of breast cancer. Pain. 1996;64(2):293-302. 144. Ehrnrooth E, Grau C, Zachariae R, et al. Randomized trial of opioids versus tricyclic antidepressants for radiation-induced mucositis pain in head and neck cancer. Acta Oncologica. 2001;40(6):745-750. 145. Kautio AL, Haanpää M, Saarto T, et al. Amitriptyline in the treatment of chemotherapy-induced neuropathic symptoms. J Symptom Manage. 2008;35(1):31-39. 146.Preskorn SH, Irwin HA. Toxicity of tricyclic antidepressants— kinetics, mechanism, intervention: a review. J Clin Psychiatry. 1982. 147. Sindrup SH, Gram LF, Brøsen K, et al. The selective serotonin reuptake inhibitor paroxetine is effective in the treatment of diabetic neuropathy symptoms. Pain. 1990;42(2):135-144. 148. Sindrup SH, Bach FW, Madsen C, et al. Venlafaxine versus imipramine in painful polyneuropathy: a randomized, controlled trial. Neurology. 2003;60(8):1284-1289. 149. Tasmuth T, Härtel B, Kalso E. Venlafaxine in neuropathic pain following treatment of breast cancer. Eur J Pain. 2002;6(1):17-24. 150. Smith EM, Pang H, Cirrincione C, et al; Alliance for Clinical Trials in Oncology. Effect of duloxetine on pain, function, and quality of life among patients with chemotherapy-induced painful peripheral neuropathy: a randomized clinical trial. JAMA 2013;309(13):1359-1367. 151. Semenchuk MR, Sherman S, Davis B. Double-blind, randomized trial of bupropion SR for the treatment of neuropathic pain. Neurology. 2001;57(9):1583-1588. 152. Smith P, Bruera E. Management of malignant ureteral obstruction in the palliative care setting. J Pain Symptom Manage. 1995;10(6):481-486. 153. Yennurajalingam S, Frisbee-Hume S, Palmer JL, et al. Reduction of cancer-related fatigue with dexamethasone: a double-blind, randomized, placebo-controlled trial in patients with advanced cancer. J Clin Oncol. 2013;31(25):3076-3082. 154. Heimdal K, Hirschberg H, Slettebø H, et al. High incidence of serious side effects of high-dose dexamethasone treatment in patients with epidural spinal cord compression. J Neuro-Oncology. 1992;12(2):141-144. 155. Sørensen S, Helweg-Larsen S, Mouridsen H, Hansen HH. Effect of high-dose dexamethasone in carcinomatous metastatic spinal cord compression treated with radiotherapy: a randomised trial. Eur J Cancer. 1994;30(1):22-27. 156. Vecht CJ, Hovestadt A, Verbiest H, et al. Dose-effect relationship of dexamethasone on Karnofsky performance in metastatic brain tumors: a randomized study of doses of 4, 8, and 16 mg per day. Neurology. 1994;44(4):675. 157. Loblaw DA, Wu JS, Kirkbride P, et al. Pain flare in patients with bone metastases after palliative radiotherapy—a nested randomized control trial. Support Care Cancer. 2007;15(4):451-455. 158. Jensen TS. Anticonvulsants in neuropathic pain: rationale and clinical evidence. Eur J Pain. 2002;6(suppl A):61-68. 159. Caraceni A, Zecca E, Bonezzi C, et al. Gabapentin for neuropathic cancer pain: a randomized controlled trial from the Gabapentin Cancer Pain Study Group. J Clin Oncol. 2004;22(14):2909-2917. 160. Sabers A, Gram L. Newer anticonvulsants. Drugs. 2000;60(1):23-33. 161. Yajnik S, Singh GP, Singh G, et al. Phenytoin as a coanalgesic in cancer pain. J Pain Symptom Manage. 1992;7(4):209-213. 162. Dunteman ED. Levetiracetam as an adjunctive analgesic in neoplastic plexopathies: case series and commentary. J Pain Palliat Care Pharmacother. 2005;19(1):35-43. 163. Rao RD, Flynn PJ, Sloan JA, et al. Efficacy of lamotrigine in the management of chemotherapy-induced peripheral neuropathy. Cancer. 2008;112(12):2802-2808. 164. Bruera E, Ripamonti C, Brenneis C, et al. A randomized doubleOctober 2015, Vol. 22, No. 4 blind crossover trial of intravenous lidocaine in the treatment of neuropathic cancer pain. J Pain Symptom Manage. 1992;7(3):138-140. 165. Sharma S, Rajagopal MR, Palat G, et al. A phase II pilot study to evaluate use of intravenous lidocaine for opioid-refractory pain in cancer patients. J Pain Symptom Manage. 2009;37(1):85-93. 166. Ripamonti C, Bruera E. Pain and symptom management in palliative care. Cancer Control. 1996;3(3):204-213. 167. Sloan P, Basta M, Storey P, et al. Mexiletine as an adjuvant analgesic for the management of neuropathic cancer pain. Anesthesia Analgesia. 1999;89(3):760. 168. Wallace MS, Magnuson S, Ridgeway B. Efficacy of oral mexiletine for neuropathic pain with allodynia: a double-blind, placebo-controlled, crossover study. Reg Anesth Pain Med. 2000;25(5):459-467. 169. Galer BS, Rowbotham MC, Perander J, et al. Topical lidocaine patch relieves postherpetic neuralgia more effectively than a vehicle topical patch: results of an enriched enrollment study. Pain. 1999;80(3):533-538. 170.Gammaitoni AR, Davis MW. Pharmacokinetics and tolerability of lidocaine patch 5% with extended dosing. Ann Pharmacother. 2002;36(2):236-240. 171. Parsons CG. NMDA receptors as targets for drug action in neuropathic pain. Eur J Pharmacol. 2001;429(1):71-78. 172. Mercadante S, Arcuri E, Tirelli W, et al. Analgesic effect of intravenous ketamine in cancer patients on morphine therapy: a randomized, controlled, double-blind, crossover, double-dose study. J Pain Symptom Manage. 2000;20(4):246-252. 173. Sinner B, Graf BM. Ketamine. Modern Anesthetics. 2008:313-333. 174. Sikora K, Advani S, Koroltchouk V, et al. Essential drugs for cancer therapy: a World Health Organization consultation. Ann Oncol. 1999;10(4):385-390. 175. Schmid RL, Sandler AN, Katz J. Use and efficacy of low-dose ketamine in the management of acute postoperative pain: a review of current techniques and outcomes. Pain. 1999;82(2):111-125. 176. Okon T. Ketamine: an introduction for a pain and palliative medicine physician. Pain Physician. 2007;10(3):493. 177. Hardy J, Quinn S, Fazekas B, et al. Randomized, double-blind, placebo-controlled study to assess the efficacy and toxicity of subcutaneous ketamine in the management of cancer pain. J Clin Oncol. 2012;30(29):3611-3617. 178. Johnson JR, Potts R. Cannabis-based medicines in the treatment of cancer pain: a randomised, double-blind, parallel group, placebo controlled, comparative study of the efficacy, safety and tolerability of Sativex and Tetranabinex in patients with cancer-related pain. British Pain Society; March 8–11, 2005; Edinburgh, Scotland. 179. Johnson JR, Burnell-Nugent M, Lossignol D, et al. Multicenter, double-blind, randomized, placebo-controlled, parallel-group study of the efficacy, safety, and tolerability of THC: CBD extract and THC extract in patients with intractable cancer-related pain. J Pain Symptom Manage. 2010;39(2):167-179. 180. Perez J. Combined cannabinoid therapy via an oromucosal spray. Drugs Today (Barc). 2006;42(8):495-503. 181. Schreiber S, Getslev V, Backer MM, et al. The atypical neuroleptics clozapine and olanzapine differ regarding their antinociceptive mechanisms and potency. Pharmacol Biochem Behav. 1999;64(1):75-80. 182. Khojainova N, Santiago-Palma J, Kornick C, et al. Olanzapine in the management of cancer pain. J Pain Symptom Manage. 2002;23(4):346-350. 183. Prommer EE. Toxicity of bisphosphonates. J Palliat Med. 2009; 12(11):1061-1065. 184. Rosen LS, Gordon D, Kaminski M, et al. Zoledronic acid versus pamidronate in the treatment of skeletal metastases in patients with breast cancer or osteolytic lesions of multiple myeloma: a phase III, double-blind, comparative trial. Cancer J. 2000;7(5):377-387. 185. Saad F, Gleason DM, Murray R, et al. A randomized, placebo-controlled trial of zoledronic acid in patients with hormone-refractory metastatic prostate carcinoma. J Natl Cancer Inst. 2002;94(19):1458-1468. 186. Berenson JR, Lichtenstein A, Porter L, et al. Efficacy of pamidronate in reducing skeletal events in patients with advanced multiple myeloma. N Engl J Med. 1996;334(8):488-493. 187. Rosen LS, Gordon D, Tchekmedyian S, et al; Zoledronic Acid Lung Cancer and Other Solid Tumors Study Group. Zoledronic acid versus placebo in the treatment of skeletal metastases in patients with lung cancer and other solid tumors: a phase III, double-blind, randomized trial. J Clin Oncol. 2003;21(16):3150-3157. 188. Prommer E. Palliative oncology denosumab. Am J Hospice Palliat Care. 2015;32(5):568-572. 189. Lewington VJ, McEwan AJ, Ackery DM, et al. A prospective, randomised double-blind crossover study to examine the efficacy of strontium-89 in pain palliation in patients with advanced prostate cancer metastatic to bone. Eur J Cancer Clin Oncol. 1991;27(8):954-958. 190. Penn RD, Paice JA, Kroin JS. Octreotide: a potent new non-opiate analgesic for intrathecal infusion. Pain. 1992;49(1):13-19. 191. Bernard SA, Bruera E. Drug interactions in palliative care. J Clin Oncol. 2000;18(8):1780-1799. October 2015, Vol. 22, No. 4 Cancer Control 425 Pharmacogenomic data may help guide treatment options for opioid use in patients with cancer-related pain, but test results should be available at the point of care. Photo courtesy of Lisa Scholder. Greener Pastures. 16" × 22". Clinical Implications of Opioid Pharmacogenomics in Patients With Cancer Gillian C. Bell, PharmD, Kristine A. Donovan, PhD, and Howard L. McLeod, PharmD Background: Pain can be a significant burden for patients with cancer and may have negative effects on their quality of life. Opioids are potent analgesics and serve as a foundation for pain management. The variation in response to opioid analgesics is well characterized and is partly due to genetic variability. Methods: We reviewed the results of clinical studies to evaluate the relationships between genetic variants and select genes involved in the pharmacokinetics and pharmacodynamics of opioids, with an emphasis on patients with cancer. Results: In patients with cancer-related pain, genetic variation in OPRM1, COMT, and ABCB1 is associated with response to morphine, which is the most well-studied opioid. Although it has not been studied in patients with cancer-related pain, the effect of CYP2D6 variation is well characterized with codeine and tramadol. Evidence is limited for associating the genetic variation and pain response of oxycodone, hydrocodone, and fentanyl in patients with cancer. Conclusion: The clinical availability of pharmacogenomic testing and research findings related to these polymorphic genes suggest that genotyping patients for these genetic variants may allow health care professionals to better predict patient response to pain and, thus, personalize pain treatment. Introduction Pain is one of the most burdensome symptoms associated with cancer and its treatment and is estimated to affect 49% to 57% of patients with curable cancer From the DeBartolo Family Personalized Medicine Institute (GCB, HLM) and the Departments of Population Sciences (GCB, HLM) and Supportive Care Medicine (KAD), H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida. Submitted June 18, 2015; accepted September 8, 2015. Address correspondence to Gillian C. Bell, PharmD, Mission Health, Fullerton Genetics Center, 9 Vanderbilt Park Drive, Asheville, NC 28803. E-mail: Gillian.Bell@msj.org Dr McLeod has disclosed that he is a consultant for Cancer Genetics. No significant relationships exist between the other authors and the companies/organizations whose products or services may be referenced in this article. Dr Bell is now affiliated with Mission Health, Personalized Medicine Program, Asheville, North Carolina. 426 Cancer Control and 56% to 75% of patients with advanced disease.1-3 Uncontrolled pain can have significant adverse effects on function, mood, sleep, and quality of life, and studies suggest that pain is an independent prognostic factor for overall survival in cancer.4-7 A study of 2,761 patients with cancer reported that one-third of patients with initial pain had a significant reduction in pain at 1 month and one-fifth had an increase in pain scores.8 Opioids are the most potent analgesics available and remain the cornerstone of clinical pain management.9 The choice of opioid analgesic depends on a number of medical and nonmedical factors.10 Nonopioid and co-analgesics (eg, nonsteroidal anti-inflammatory drugs), as well as nonpharmacological measures, are often used to improve analgesic control, reduce opioid requirements, or both, as well as minimize adverse events related to opioid use.11 ReOctober 2015, Vol. 22, No. 4 gardless of the analgesic type, effective pain management depends on achieving a favorable balance between adequate analgesia and adverse events. It is well documented that patients vary considerably in their response to pain therapies, including medication.12 Response in analgesia and to adverse events may vary between patients with similar pain levels or disease status and may vary among patients at different stages of the care trajectory.9,10,13 Increasingly, research has been aimed at identifying the hereditary basis for interindividual differences in drug effects to explain altered efficacy and adverse events.12 Currently, genetic factors may be responsible for 12% to 60% of response variability in opioid therapy.14 Using genetic variation to help guide drug therapy choices may be helpful in balancing the exclusion of low-yield therapy, avoiding adverse events, and achieving pain control. Genes Modulating Opioid Response Many genes have been studied to identify pharmacogenomic markers in opioid therapy, including genes implicated in the pharmacodynamics (OPRM1, COMT) and pharmacokinetics (CYP2D6, CYP3A4/5, ABCB1) of opioids.14,15 Pharmacokinetics Transporters One of the most studied transporters in opioids is a member of the adenosine triphosphate–binding cassette, sub-family B, member 1 (ABCB1), also known as P-glycoprotein or multidrug resistance protein 1. ABCB1 transporters are present in numerous locations, including the gastrointestinal tract, liver, kidneys, and the blood–brain barrier, and they facilitate the absorption, distribution, and elimination of medications, including opioids such as morphine and fentanyl and their metabolites.15 Impairment of these transporters may result in increased bioavailability of oral medications, decreased renal excretion, and increased central nervous system concentrations.16 Specifically, the transport of opioids into the brain through the blood–brain barrier may be affected by variations in ABCB1 transporters at this site. ABCB1 is highly polymorphic, with more than 100 single nucleotide polymorphisms (SNPs) identified, but C3435T (rs1045642) is the most widely studied. The 3435T variant is associated with decreased mRNA expression, protein expression, or both in some tissues.14 Although the results have been mixed, several studies report a difference in pain relief and opioid doses between reference and variant genotypes.16-18 Cytochrome P450 Enzymes Cytochrome P450s are a gene superfamily of catalytic proteins that fill important roles across the specOctober 2015, Vol. 22, No. 4 trum of cellular biochemical reactions, including endogenous hormone production and xenobiotic metabolism. This includes the activation of many opioids from a relatively inert compound to a pharmacologically active molecule. The enzyme CYP3A4 plays a role in the metabolism of numerous medications, including many opioids (eg, methadone, oxycodone, hydrocodone, fentanyl), but few studies link genetic variations to opioid response.14 The cytochrome P450 enzyme 2D6 (CYP2D6) influences the metabolism of 25% of all drug therapies, including codeine, hydrocodone, oxycodone, and tramadol, as well as tricyclic antidepressants.12,19 Genetic variation in the CYP2D6 enzyme is one of the most studied and wellunderstood of all the drug-metabolizing enzymes.20 The effect of variation on phenotype is typically classified into 4 major groups: poor metabolizers (5%–10%), intermediate metabolizers (2%–11%), extensive metabolizers (77%–92%), and ultra-rapid metabolizers (1%–2%).21 These percentages are based on data for whites and will vary for other ethnicities.21 Reports of therapeutic failure (lack of pain control, opioid-related adverse events, or both) in patients with specific genotypes receiving select opioids (codeine, hydrocodone, oxycodone, tramadol, fentanyl, methadone) suggest a significant impact of CYP2D6 genetic variants on drug efficacy and adverse-event profile.12,22 In addition, CYP2D6 metabolizer status becomes particularly important when concomitant medications affecting other pharmacokinetic pathways are used. Serious or fatal, inadvertent codeine overdose can occur when a patient with cancer and CYP2D6 ultrarapid metabolizer status is also treated with CYP3A4 inhibitors, such as clarithromycin and voriconazole.23 The “double hit” of hyperactivation to morphine via CYP2D6 and the reduced inactivation due to blocked CYP3A4 leads to life-threatening respiratory depression.24 Pharmacodynamics OPRM1 The µ-opioid receptor gene encoded by the genetic locus OPRM1 is the primary binding site for endogenous opioid peptides and opioid analgesics.12 As such, OPRM1 is a biologically plausible candidate for evaluating the role of polymorphism in the clinical effects of opioids.19 More than 100 SNPs have been described for OPRM1.25 The most prevalent and widely studied SNP is a nonsynonymous nucleotide substitution at position 118 (A118G; rs1799971). The frequency of this SNP widely varies among different races and ethnicities: 4.7% in Africans, 15.4% in Europeans, 48.5% in Japanese, and 14% in Hispanics.26 To assess the possible functional effect of each allele of this gene, persons are identified by 1 of 3 genotypes: homozygous G/G, homozygous A/A, or heterozygous G/A. Cancer Control 427 This SNP has been associated with variation in opioid response in a number of settings, including cancerrelated pain.27 Numerous studies have examined the relationship of OPRM1 genetic variants to pain control in the oncology setting with mixed results.17,28-31 variant in relation to pain response, adverse events, or both in patients with cancer and have had mixed results.15 Other variants commonly studied include COMT Val158Met and ABCB1 C3435T.15 One study evaluated the relationship of 4 OPRM1 polymorphisms and pain control in 207 study patients with cancer pain.29 A total of 99 volunteers who completed the Brief Pain Inventory (BPI) survey and had adequate pain control were included in the final analysis.29 The A118G variant alone was related to morphine dose. Participants with G/G genotype (225 ± 143 mg/24 hours) required significantly higher doses of morphine when compared with participants with wild-type A/A (97 ± 89 mg/24 hours) and A/G (66 ± 50 mg/24 hours) COMT COMT encodes an enzyme involved in the metabolism of catecholamines, including epinephrine, norepinephrine, and dopamine, which play a role in pain modulation.32 Impairment of the catechol-O-methyltransferase (COMT) enzyme, which results in increased concentrations of dopamine, can suppress the production of endogenous opioids (eg, enkephalin), which, in turn, cause subsequent opioid receptor expression upregulation.16 Table. — Summary of Select Genetic Variants Associated The COMT gene locus contains With Response in Cancer Pain multiple SNPs; the most studied SNP is Val158Met, also known as Opioid Study Genetic No. of Results Agent Variant Patients rs4680.33 It has been postulated that this polymorphism leads to a Fentanyl ReyesOPRM1 A118G 695 No difference in dose requirements for Gibby 28 all variants 3- to 4-fold reduced activity of the COMT Val158Met COMT enzyme and has been assoABCB1 C3435T ciated in patients with cancer with CYP3A5*3 60 Greater central adverse events with Ross 36 *3/*3 genotype increased sensitivity to painful ABCB1 C1236T stimuli (for the Val/Val genotype) COMT Val158Met 221 No difference in central adverse events Morphine Belfer 33 and with lower doses of morphine (confusion, drowsiness, hallucination) ABCB1 C3435T required for satisfactory relief Other COMT and ABCB1 variants associated with central adverse events of pain (for the Met/Met geno156 No difference in response Branford27 OPRM1 A118G type).28,33-35 Variation in COMT may also affect unwanted effects of opi99 Patients with G/G genotype required Kasai and OPRM1 A118G higher doses of morphine when comIkeda 26 oid treatment such as sedation.36 pared with A/G and A/A genotypes A limited number of studies 16 OPRM1 A118G 137 Patients with OPRM1 A/A identified as Lotsch have examined the relationship having good responses indicated by ABCB1 C3435T of variants in these genes to pain decrease in numerical rating scale control in cancer, and nearly all Presence of ≥ 1 OPRM1 G allele were of these studies have involved the found have worst response regardless of ABCB1 genotype acute postoperative period with Patients with OPRM1 A/A and ABCB1 outcomes related to cancer recurT/T best responders with greatest rence and progression.12,27 Few decrease in scores studies have involved patients 207 Patients with OPRM1 A/A and COMT Ravindra- OPRM1 A118G with cancer, and patient-reported Met/Met combined required lowest nathan25 COMT Val158Met morphine dose when compared with pain outcomes in relation to these other combinations of genotypes genetic variants and pain relief is OPRM1 A118G 827 No difference in dose requirements for Reyessparingly used.15 Commonly used all variants Gibby 28 COMT Val158Met opioids in patients with cancer ABCB1 C3435T and the genetic variants reportCOMT Val158Met 207 Patients with Val/Val genotype Ross 31 ed to modulate response are rerequired higher dose of morphine comviewed in the Table.14,16,25-28,31,33,36 pared with Val/Met and Met/Met Pharmacogenomics Morphine Because the µ-opioid receptor is the main site of action for morphine, numerous studies have evaluated the OPRM1 A118G 428 Cancer Control Oxycodone Hajj14 CYP2D6 450 No difference in pain intensity or adverse events (nausea, sedation, cognition) between metabolizer status ReyesGibby 28 OPRM1 A118G COMT Val158Met ABCB1 C3435T 445 No difference in dose requirements for all variants October 2015, Vol. 22, No. 4 genotypes.29 Those with the A/G genotype also had the highest average BPI scores, a finding pointing to the possible undertreatment of pain, which might explain the lower doses administered to those with that genotype.29 In addition, the authors found no differences in adverse-event intensity between the groups.29 A subsequent study reanalyzed the same 207 volunteers to look for a relationship between the COMT variant Val158 Met polymorphism and morphine requirements.34 In this study, all participants were included in the analysis. Those with the Val/Val genotype received the highest dose of morphine (155 ± 160 mg/24 hours) followed by those with the Val/Met genotype (117 ± 100 mg/24 hours) and the Met/Met genotype (95 ± 99 mg/24 hour; P = .025).34 Reyes-Gibby et al28 conducted a secondary analysis combining the results of the above-mentioned studies in the same patients to explore the joint effects of COMT Val158Met and OPRM1 A118G variants on the efficacy of morphine for cancer pain. Carriers of both wild-type OPRM1 A/A and COMT Met/Met genotypes had the lowest morphine dose (87 mg/24 hours; 95% confidence interval [CI]: 57–116), which significantly differed from study participants with neither Met/Met nor A/A who had the highest dose (147 mg/24 hours; 95% CI: 100–180).28 After controlling for demographic and clinical variables, such as age, sex, time from cancer diagnosis, and months using morphine, the joint-effect results remained.28 Campa et al17 evaluated the relationship of OPRM1 A118G and ABCB1 C3435T variants to pain response in 137 study patients with cancer receiving treatment with morphine. Pain response was measured using an 11-point numerical rating scale (NRS) and the main end point was a change in NRS at the end of the first 7 days.17 At the end of the first 7 days, study patients with wild-type OPRM1 A/A genotype had a significantly greater change in NRS score (3.73; standard deviation [SD] ± 1.72) when compared with homozygous-variant study patients (0.30; SD ± 1.77).17 In addition, study patients with ABCB1 T/T genotype had a significantly greater change in NRS score (4.39; SD ± 2.21) than homozygous wild-type study patients (2.31; SD ± 1.73).17 Multivariate analysis assessed the joint effects of variation in the 2 genes. Study patients with at least 1 OPRM1 variant were the worst responders regardless of ABCB1 genotype; conversely, study patients with wild-type OPRM1 A/A genotype had a good response.17 Study patients with wild-type OPRM1 A/A and homozygous-variant ABCB1 T/T genotype were the best responders, with an NRS score of 4.8 ± 1.62.17 Some studies report lack of association with numerous genetic variants.30,31,36 An observational, single time point trial with 156 study patients with cancer found no association with the OPRM1 A118G variant October 2015, Vol. 22, No. 4 and response to morphine.30 This study evaluated numerous genetic variants in 4 genes (OPRM1, ARRB2, STAT6, and UGT2B7) and consisted of morphine responders (controlled on morphine for ≥ 1 month) and nonresponders (switched alternative agents due to inadequate analgesia with titrated doses or intolerable adverse events).30 No association was found with variants in OPRM1 and UGT2B7, but numerous variants in the other 2 genes were associated with response.30 One of the largest trials to date evaluated the influence of 112 genetic variants of 23 candidate genes on the efficacy of numerous opioids in 2,201 study patients with cancer: morphine (n = 827), oxycodone (n = 445), fentanyl (n = 695), and other opioids (n = 234).31 All drug doses were converted to equivalent morphine doses and pain intensity was measured using the BPI.31 Klepstad et al31 noted that all SNPs, including OPRM1 A118G, ABCB1 C3435T, and COMT Val158Met, failed to show a relationship with opioid dose. A case-control study of 221 participants with cancer treated with morphine reported no association with COMT Val158Met or ABCB1 C343T variants and the central adverse events of confusion, drowsiness, and hallucination.36 Of note, other variants in COMT and ABCB1 were associated with central adverse events.36 Because of these conflicting data, concluding whether these variants predict morphine analgesia or adverse events is difficult. The most benefit will likely be derived from combining these markers to identify patients with a poor-response profile for which an alternative therapy may be preferred. Codeine The analgesic properties of codeine are derived from its conversion to morphine and morphine-6-glucoronide by CYP2D6 (Fig).21 Persons who are CYP2D6 poor metabolizers have little or no CYP2D6 enzyme activity and will not attain a meaningful degree of pain control. An important safety concern is that persons with extra copies of CYP2D6 convert codeine to morphine to a greater extent and may be at risk for adverse events such as sedation and even respiratory depression.21 The results of numerous studies (mainly in patients without cancer) suggest a difference in analgesia and adverse effects across CYP2D6 metabolizer statuses and these differences have been highlighted in a peer-reviewed guideline by the Clinical Pharmacogenetics Implementation Consortium.21 Therefore, CYP2D6 testing is useful in determining which patients will derive the most benefit from codeine use as well as patients who may be at increased risk for toxicity. Tramadol Similar to codeine, CYP2D6 is responsible for the conversion of tramadol to O-desmethyltramadol, which has a 200-fold greater affinity for the µ-opioid Cancer Control 429 UGT1A1 UGT2B7 Codeine CYP2D6 UGT1A1 UGT2B7 Morphine CYP2D6 Oxycodone Noroxycodone CYP2D6 Fentanyl Tramadol Hydromorphone CYP3A4 CYP3A4/5 CYP2D6 Morphine-3-glucoronide Oxymorphone CYP3A4 Hydrocodone Morphine-6-glucoronide Norhydrocodone Norfentanyl O-desmethyltramadol Fig. — Metabolic pathways of commonly used opioids. receptor target (see Fig).21 Several studies have reported that CYP2D6 poor metabolizers display decreased analgesic response with tramadol than those who are extensive metabolizers.37-39 No studies have focused on the pharmacogenomics of tramadol specifically in a cancer population; however, given the evidence in other pain populations, tramadol is likely to have reduced clinical benefit in patients who poorly metabolize CYP2D6.37-39 Oxycodone The majority of oxycodone is metabolized to noroxycodone by CYP3A4 (see Fig).40 A smaller percentage (11%) is converted by CYP2D6 to the active metabolite oxymorphone, which has a 40-fold higher affinity and 8-fold higher potency for μ-opioid receptors than oxycodone.21 Studies evaluating CYP3A4 variation to oxycodone response are scarce, whereas studies in healthy volunteers and postoperative patients have reported mixed results on CYP2D6 polymorphisms and response to oxycodone.15 In a cross-sectional study of 450 study patients with cancer treated with oxycodone, Andreassen et al22 reported no difference in pain intensity using the BPI or the incidence of adverse events 430 Cancer Control (nausea, sedation, cognitive) between CYP2D6 metabolizer statuses. In the large study of 2,201 study patients with cancer being treated with various opioids, including 445 of whom were treated with oxycodone, Klepstad et al31 could not determine an association with OPRM1, ABCB1, and COMT variants (as well as numerous other genes) and opioid requirements. Based on this evidence, little basis exists for the use of pharmacogenomics to personalize the use of oxycodone therapy in patients with cancer. Hydrocodone Hydrocodone is metabolized by CYP2D6 to the active metabolite hydromorphone, which has a 10- to 33-fold greater affinity for μ-opioid receptors than hydrocodone (see Fig).40 Additional metabolism includes the formation of norhydrocodone by CYP3A4, and 40% of clearance is attributed to non-CYP pathways.40 No studies have focused on the pharmacogenomics of hydrocodone specifically in a cancer population. Thus, little basis exists for the use of pharmacogenomics to personalize the use of hydrocodone therapy in patients with cancer. October 2015, Vol. 22, No. 4 Fentanyl Fentanyl is primarily metabolized by CYP3A4/5 to the inactive metabolite norfentanyl (see Fig).15 Most of the current literature assessing the effect of CYP3A4/5 on pain outcomes involves the postoperative patient population, and a robust association has not yet been discovered.15 Klepstad et al 31 studied 2,201 volunteers with cancer being treated with various opioids, including 695 of whom were treated with fentanyl, and found no association with OPRM1, ABCB1, and COMT variants (as well as numerous other genes) and opioid requirements.31 A small trial of 60 Asian study patients with cancer who were treated with transdermal fentanyl reported more and greater-intensity central adverse events with patients homozygous for CYP3A5*3 when compared with patients with *1/*1 and *1/*3 genotypes.41 The authors also studied 3 ABCB1 variants (C1236T, G2677A/T, and C3435T), and C1236T alone was associated with response with decreased administration of rescue medication reported in the homozygous T/T variant.41 To date, the utility of CYP3A4/5 and ABCB1 testing to personalize fentanyl dosing is uncertain. Implementation in Clinical Practice The clinical availability of pharmacogenomics testing and the research findings related to these polymorphic genes suggest that genotyping patients for some of these genetic variants may help predict response to pain treatment with good rates of sensitivity and specificity and with greater benefits for patients and decreased health care utilization.17 One key element of using pharmacogenomics for the treatment of cancer pain is employing a preemptive testing strategy so the results will be available at the point of care. It is crucial to have information on individual patient pharmacogenomics at the time of a care decision so that the data can be used to guide therapeutic decision-making. As part of this preemptive strategy, these results must be readily retrievable. In addition, use of clinical-decision support alerts has been shown to inform clinicians of important genetic results and guide treatment at the point of care.42 Conclusions Opioids are heavily used in the treatment of patients with cancer-related pain, and individual genetic variation affects the pharmacokinetics and pharmacodynamics of opioids.9 The evidence linking commonly used opioids has been reviewed for patients with cancer and the genetic variants reported to modulate response and results. Although the results seem promising, prospective studies randomizing patients to genotype-guided pain management compared with standard practice are needed. In addition, further evaluation of complex interactions (drug–gene–gene and October 2015, Vol. 22, No. 4 drug–drug–gene) must be explored. If pharmacogenomics data are used to guide treatment decisions, then a preemptive testing strategy is a key element that must be included and test results must be available at the point of care. 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Randomized clinical trial of an implantable drug delivery system compared with comprehensive medical management for refractory cancer pain: impact on pain, drug-related toxicity, and survival. J Clin Oncol. 2002;20(19):4040-4049. 7. Herndon JE II, Fleishman S, Kornblith AB, et al. Is quality of life predictive of the survival of patients with advanced nonsmall cell lung carcinoma? Cancer. 1999;85(2):333-340. 8. Zhao F, Chang VT, Cleeland C, et al. Determinants of pain severity changes in ambulatory patients with cancer: an analysis from Eastern Cooperative Oncology Group trial E2Z02. J Clin Oncol. 2014;32(4):312-319. 9. Mercadante S, Porzio G, Gebbia V. New opioids. J Clin Oncol. 2014;32(16):1671-1676. 10. Slatkin NE. Opioid switching and rotation in primary care: implementation and clinical utility. Curr Med Res Opin. 2009;25(9):2133-2150. 11. Vardy J, Agar M. Nonopioid drugs in the treatment of cancer pain. J Clin Oncol. 2014;32(16):1677-1690. 12. Kleine-Brueggeney M, Musshoff F, Stuber F, et al. Pharmacogenetics in palliative care. Forensic Sci Int. 2010;203(1-3):63-70. 13. Wiffen PJ, Derry S, Moore RA. Impact of morphine, fentanyl, oxycodone or codeine on patient consciousness, appetite and thirst when used to treat cancer pain. Cochrane Database Syst Rev. 2014;5:CD011056. 14. Hajj A, Khabbaz L, Laplanche JL, et al. Pharmacogenetics of opiates in clinical practice: the visible tip of the iceberg. Pharmacogenomics. 2013;14(5):575-585. 15. Nielsen LM, Olesen AE, Branford R, et al. Association between human pain-related genotypes and variability in opioid analgesia: an updated review. Pain Pract. 2015;15(6):580-594. 16.Lötsch J, von Hentig N, Freynhagen R, et al. Cross-sectional analysis of the influence of currently known pharmacogenetic modulators on opioid therapy in outpatient pain centers. Pharmacogenet Genomics. 2009;19(6):429-436. 17. Campa D, Gioia A, Tomei A, et al. Association of ABCB1/MDR1 and OPRM1 gene polymorphisms with morphine pain relief. Clin Pharmacol Ther. 2008;83(4):559-566. 18. Hung CC, Chiou MH, Huang BH, et al. Impact of genetic polymorphisms in ABCB1, CYP2B6, OPRM1, ANKK1 and DRD2 genes on methadone therapy in Han Chinese patients. Pharmacogenomics. 2011;12(11):1525-1533. 19. Smith MT, Muralidharan A. Pharmacogenetics of pain and analgesia. Clin Genet. 2012;82(4):321-330. 20. Rollason V, Samer C, Piguet V, et al. Pharmacogenetics of analgesics: toward the individualization of prescription. Pharmacogenomics. 2008;9(7):905-933. 21. Crews KR, Gaedigk A, Dunnenberger HM, et al; Clinical Pharmacogenetics Implementation Consortium. Clinical Pharmacogenetics Implementation Consortium guidelines for cytochrome P450 2D6 genotype and codeine therapy: 2014 update. Clin Pharmacol Ther. 2014;95(4):376-382. 22. Andreassen TN, Eftedal I, Klepstad P, et al. Do CYP2D6 genotypes reflect oxycodone requirements for cancer patients treated for cancer pain? A cross-sectional multicentre study. Eur J Clin Pharmacol. 2012;68(1):55-64. 23. Gasche Y, Daali Y, Fathi M, et al. Codeine intoxication associated with ultrarapid CYP2D6 metabolism. N Engl J Med. 2004;351(27):2827-2831. 24. Wilkinson GR. Drug metabolism and variability among patients in drug response. N Engl J Med. 2005;352(21):2211-2221. 25. Ravindranathan A, Joslyn G, Robertson M, et al. Functional characterization of human variants of the mu-opioid receptor gene. Proc Natl Acad Sci U S A. 2009;106(26):10811-10816. Cancer Control 431 26. Kasai S, Ikeda K. Pharmacogenomics of the human micro-opioid receptor. Pharmacogenomics. 2011;12(9):1305-1320. 27. Branford R, Droney J, Ross JR. Opioid genetics: the key to personalized pain control? Clin Genet. 2012;82(4):301-310. 28. Reyes-Gibby CC, Shete S, Rakvåg T, et al. Exploring joint effects of genes and the clinical efficacy of morphine for cancer pain: OPRM1 and COMT gene. Pain. 2007;130(1-2):25-30. 29. Klepstad P, Rakvåg TT, Kaasa S, et al. The 118 A > G polymorphism in the human mu-opioid receptor gene may increase morphine requirements in patients with pain caused by malignant disease. Acta Anaesthesiol Scand. 2004;48(10):1232-1239. 30. Ross JR, Rutter D, Welsh K, et al. Clinical response to morphine in cancer patients and genetic variation in candidate genes. Pharmacogenomics J. 2005;5(5):324-336. 31. Klepstad P, Fladvad T, Skorpen F, et al; European Palliative Care Research Collaborative (EPCRC); European Association for Palliative Care Research Network. Influence from genetic variability on opioid use for cancer pain: a European genetic association study of 2294 cancer pain patients. Pain. 2011;152(5):1139-1145. 32. Andersen S, Skorpen F. Variation in the COMT gene: implications for pain perception and pain treatment. Pharmacogenomics. 2009;10(4):669-684. 33. Belfer I, Segall S. COMT genetic variants and pain. Drugs Today (Barc). 2011;47(6):457-467. 34. Rakvåg TT, Klepstad P, Baar C, et al. The Val158Met polymorphism of the human catechol-O-methyltransferase (COMT) gene may influence morphine requirements in cancer pain patients. Pain. 2005;116(1-2):73-78. 35. Rakvåg TT, Ross JR, Sato H, et al. Genetic variation in the catechol-O-methyltransferase (COMT) gene and morphine requirements in cancer patients with pain. Mol Pain. 2008;4:64. 36. Ross JR, Riley J, Taegetmeyer AB, et al. Genetic variation and response to morphine in cancer patients: catechol-O-methyltransferase and multidrug resistance-1 gene polymorphisms are associated with central side effects. Cancer. 2008;112(6):1390-1403. 37. Stamer UM, Musshoff F, Kobilay M, et al. Concentrations of tramadol and O-desmethyltramadol enantiomers in different CYP2D6 genotypes. Clin Pharmacol Ther. 2007;82(1):41-77. 38. Poulsen L, Arendt-Nielsen L, Brøsen K, et al. The hypoalgesic effect of tramadol in relation to CYP2D6. Clin Pharmacol Ther. 1996;60(6):636-644. 39. Stamer UM, Lehnen K, Höthker F, et al. Impact of CYP2D6 genotype on postoperative tramadol analgesia. Pain. 2003;105(1-2):231-238. 40. Vuilleumier PH, Stamer UM, Landau R. Pharmacogenomic considerations in opioid analgesia. Pharmgenomics Pers Med. 2012;5:73-87. 41. Takashina Y, Naito T, Mino Y, et al. Impact of CYP3A5 and ABCB1 gene polymorphisms on fentanyl pharmacokinetics and clinical responses in cancer patients undergoing conversion to a transdermal system. Drug Metab Pharmacokinet. 2012;27(4):414-421. 42. Bell GC, Crews KR, Wilkinson MR, et al. Development and use of active clinical decision support for preemptive pharmacogenomics. J Am Med Inform Assoc. 2014;21(e1):e93-e99. 432 Cancer Control October 2015, Vol. 22, No. 4 Communication between patients, their relatives, and health care staff is very important when administered palliative sedation. Photo courtesy of Lisa Scholder. Organic Walk, 16" × 24". Palliative Sedation in Patients With Cancer Marco Maltoni, MD, and Elisabetta Setola, MD Background: Palliative sedation involves the use of sedative medication to relieve refractory symptoms in patients by reducing their level of consciousness. Although it is considered an acceptable clinical practice from most ethical points of view, palliative sedation is still a widely debated procedure and merits better understanding. Methods: The relevant medical literature pertaining to palliative sedation was analyzed and reviewed from various technical, relational, and bioethical perspectives. Results: Proportionate palliative sedation is considered to be the most clinically appropriate modality for performing palliative sedation. However, guidelines must be followed to ensure that it is performed correctly. Benzodiazepines represent the first therapeutic option and careful monitoring of dosages is essential to avoid oversedation or undersedation. Conclusions: Proportionate palliative sedation is used to manage and relieve refractory symptoms in patients with cancer during their last days or hours of life. Evidence suggests that its use has no detrimental effect on survival. A different decision-making process is used to manage the withdrawal of hydration than the process used to determine whether proportionate palliative sedation is appropriate. Communication between patients, their relatives, and the health care staff is important during this medical intervention. Introduction Within the context of palliative medicine, the practice of drug-induced sedation for symptom control — the use of all possible antisymptomatic treatments notwithstanding — is called palliative sedation. Historically, palliative sedation was often known as terminal sedation because it was considered a last reFrom the Palliative Care Unit (MM), Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, Meldola, Italy, and the Oncology Unit (ES), Saint Giuseppe Hospital, Milan, Italy. Submitted April 15, 2015; accepted July 15, 2015. Address correspondence to Marco Maltoni, MD, Palliative Care Unit, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, Via P. Maroncelli 40, 47014 Meldola, Italy. E-mail: marcocesare.maltoni@irst.emr.it No significant relationships exist between the authors and the companies/organizations whose products or services may be referenced in this article. October 2015, Vol. 22, No. 4 sort in the final phase of a patient’s life. However, terminal sedation is a confusing and inappropriate term because it implies that the practice is designed to shorten life; thus, more appropriate terminology was needed and, hence, the term palliative sedation. Its implementation into everyday clinical practice has led to the use of the term in the majority of studies focusing on this topic.1 By reducing the level of consciousness, palliative sedation uses sedative medication to control refractory symptoms in patients with cancer.2 As this approach became more widespread and was acknowledged as an important part of palliative care, procedural principles were needed to avoid malpractice. The guidelines created clarified the definition and practice of palliative sedation, also underlining its inherent and intrinsic difference from euthanasia.3-10 Various guidelines Cancer Control 433 state that sedation in palliative care is a therapy mainly performed in the last days or hours of life, with an unambiguous aim, a precise procedure, and well-defined results — all of which are in contrast to those of euthanasia.9-12 In addition, use of palliative sedation does not hasten death when conducted in accordance with standard guidelines.13-15 Methods and Types Some adjunctive characteristics of palliative sedation have been included in more detailed definitions of the practice.16 For example, the definition of palliative sedation as the “intentional administration of sedative drugs in dosages and in combinations required to reduce the consciousness of a terminal patient as much as necessary to adequately relieve one or more refractory symptoms,” considers a number of specific parameters, including short-term prognosis, proportionality of the intervention, effectiveness of the sedation through adequate monitoring, and refractoriness of symptoms.16 This definition also implies that palliative sedation is not a fixed intervention; rather, it is a dynamic process that can be adapted to the needs of the patient.16 Palliative sedation can vary in terms of depth of sedation and correlated level of unconsciousness, continuity, drugs used, and rapidity of implementation (Table 1). In that sense, palliative sedation is not necessarily deep continuous sedation or continuous sedation until death, which is the last step of a progressive process. “Proportionality” Theme When symptom-guided, dose-titrated, and result-assessed, palliative sedation can be a progressive or sud- den procedure (see Table 1). Choice of sedation has a clinical basis and is oriented toward different clinical needs: rapid palliative sedation for catastrophic and acute symptoms compared with proportional sedation for progressively worsening symptoms. Thus, implementing palliative sedation is based on the suddenness of symptom appearance. Different methods of palliative sedation exist, namely, the proportional method of palliative sedation (also called proportionate palliative sedation), and sustained, deep and continuous palliative sedation from the onset, regardless of the intensity of symptoms.17-21 The latter suggests that relational continuity between patients and relatives is not necessarily a value, that the concept of hastening or not hastening death is irrelevant (given the patient’s condition), and that the process of drug titration may delay the clinical effect of sedation. We consider that the supporters of this view are in favor of administering drugs in a “standardized” way, which leads to deep sedation, and interrupting all forms of hydration and nutrition. Taking this concept to an extreme, many patients in this setting would be candidates for deep continuous sedation or continuous sedation until death. In our opinion, those in favor of using palliative sedation in this way do not consider that this view is a departure from the original practice; indeed, such an approach could be counterproductive to its large-scale implementation. By contrast, some proportional palliative sedation supporters view proportionality as a fundamental characteristic of palliative sedation and maintain that relational continuity is an important issue.6,14 They also argue that the effects of palliative sedation on refrac- Table 1. — Definitions of Terms Used in Palliative Sedation Term Definition Depth Superficial Patient conserves (at least partially) the ability to communicate with family or caregivers Deep Patient enters a state of total unconsciousness Continuity Intermittent Sedation is performed for a limited period of time for a specific symptom and discontinued if a reduction (albeit slight) in the distress caused by the symptom has been obtained Continuous Drugs are administered without interruption to obtain a persistent effect, which more frequently occurs near death and in severe cases Use of Drugs Primary palliative sedation Sedatives are used to lower the level of consciousness Secondary palliative sedation Dosage of drugs primarily used for symptom control (ie, morphine for pain or dyspnea) is increased to make use of an adverse event (eg, somnolence) to reduce the level of consciousness (not recommended) Rapidity of Administration and Effect Progressive Most common method in which the dose of sedative drugs is monitored and modified according to patient needs Sudden, rapid intervention method Also called “emergency” sedation; required for a catastrophic event such as massive bleeding, severe dyspnea, agitated delirium, or acute pain 434 Cancer Control October 2015, Vol. 22, No. 4 Prevalence and Settings The debates about the characteristics and how to define palliative sedation are reflected in the wideranging prevalence of the practice observed in the literature (1%–88%),8-10,13 which can be attributed to a number of factors: • Different health care settings Higher prevalence in inpatient acute tertiary palliative care units compared with home-care hospice programs33,34 • Different case mixes in similar settings In a comparison between 2 Italian hospices, a statistically significant difference in prevalence of sedation initially observed in the 2 populations disappeared when the groups were corrected for age and duration of stay in hospice. This was interpreted as an indication that younger patients and those admitted for acute symptoms required more palliative sedation interventions than older patients admitted to the hospice for psychosocial reasons35 • Degree of adherence to palliative sedation guidelines or level of competence and expertise of health care professionals Little experience in managing difficult symptoms may increase the prevalence of palliative sedation. The decision to initiate palliative sedation should be taken together with a palliative care specialist to ensure that all other options have October 2015, Vol. 22, No. 4 been explored.5 In the event of insufficient psychological support, the palliative care team may be at risk of burn out and, thus, might perform unnecessary palliative sedation36 • Adoption of wider or narrower definition of palliative sedation may influence prevalence However, the real frequency of palliative sedation has also been suggested to vary: possibly between 25% and 35% of all patients admitted to a hospice or palliative care unit.14,15 In home-care hospice programs, palliative sedation may be a feasible option. Mercadante et al37 reported that 13.2% of patients with cancer were sedated in their homes. Symptoms and Refractoriness Management of refractory symptoms is the single indication for use of palliative sedation.14,27 The definition of a refractory symptom, which was first proposed by Cherny and Portenoy,27 is still widely accepted: “Symptom for which all possible treatment has failed, or it is estimated that no methods are available for palliation within the time frame and the risk:benefit ratio that the patient can tolerate.”5,6,8 The concept of refractoriness has been associated with that of unbearable suffering, multidisciplinary evaluation, and a prognosis of shortterm survival.7 Prior to offering sedation, all other available medications, procedures, and interventions should be considered and offered as appropriate. When possible, discussions should take place with patients, their relatives, and their caregivers so that an agreement can be made about the action to be taken.7-10 All dimensions of the symptom (physical, psychological, social, emotional, existential/spiritual) must be taken into account and help offered for each component.7-10 Physical symptoms that are most prone to refractoriness include delirium and dyspnea; both are frequently present as death approaches, and their timely management is crucial. Pain and emesis may also become refractory, albeit this occurs less frequently.14,38-41 Palliative sedation Refractory Symptoms tory symptoms must be carefully monitored to avoid undersedation or oversedation and that the drug dosage should be individually titrated to the minimum effective dose level.6,14 Not every palliative sedation is deep continuous sedation or continuous sedation until death, and the latter approach is required in patients for whom a lower level of sedation is insufficient to control refractory symptoms.7,22-25 A survey carried out by Putman et al26 on intention and practice in US physicians reported that many of these physicians preferred the proportional method, because they did not wish to pursue full unconsciousness in patients without monitoring the effect of such sedation. Moreover, some of the first articles published on palliative sedation were based on the use of a proportional, clinical, symptom-guided form of sedation.5,6,11,27,28 This form of palliative sedation is accepted by most ethical points of view, cultural perspectives, and procedural guidelines. 8-10,28-32 ten Have and Welie12 used the term mission creep to describe the intention of shortening life — however small — by using deep sedation, concluding that such an approach cannot be considered palliative sedation. In a clinical context, mission creep occurs when a specific practice considered appropriate in a given situation is gradually extended for use with different indications, groups of patients, and different intentions. 416 (54%) Delirium Dyspnea Psychological distress Pain Vomiting Other (eg, itching, bleeding) 234 (30%) 151 (19%) 135 (17%) 37 (5%) 30 (4%) 0 100 200 300 400 500 Patients Fig 1. — Main refractory symptoms requiring palliative sedation in 774 patients from 10 studies with a total of 1,003 symptoms (some patients had > 1 refractory symptom). From Maltoni M, Scarpi E, Rosati M, et al. Palliative sedation in end-of-life care and survival: a systematic review. J Clin Oncol. 2012;30(12):1378-1383. Reprinted with permission. © 2012. American Society of Clinical Oncology. All rights reserved. Cancer Control 435 is occasionally used for intractable seizures or terminal hemorrhage (Fig 1).14,42 Psychological distress and existential suffering are complex and challenging. These terms encompass issues such as meaninglessness of life, sense of hopelessness, perception of self as a burden to others, feeling dependent on others, feeling isolated, grieving, loss of dignity and purpose, fear of death of self, or fear of the unknown. Multidimensional management directed at the physical, psychological, and existential aspects is recommended, with support provided by psychologists, psychiatrists, chaplains, ethicists, or palliative care specialists.3-10 Psychological distress and existential suffering have many peculiarities.5,8,27 Psychological distress can occur at any time during the course of a disease, and supportive interventions, psychological interventions, or both types of interventions may be ineffective and do not completely resolve the problem; however, this does not mean that the distress is refractory.43 Rather, psychological distress may not have a progressive course similar to that associated with physical symptoms; its course is often unpredictable. For these reasons, frequent meetings with the palliative care team are needed; the same may be true of spiritual assistance. Intermittent or relief sedation, rather than continuous sedation, may also be beneficial.5,8,27 Some authors have focused on the search for early determinants for continuous palliative sedation so that patients at risk can be identified in a timely manner and their symptoms managed by other strategies.33,44-46 Information on determinants that more frequently lead to use of palliative sedation could also be used to assess the need for advanced care planning. However, study results have suggested that implementing palliative sedation is more often linked to the attitudes and beliefs of physicians and to organizational settings than to the clinical needs of patients.44 Other studies found a correlation between palliative sedation and higher doses of opioids used prior to implementing palliative sedation as well as between palliative sedation and younger patient age.33,45,46 Drugs The most widely used drug for palliative sedation in the context of palliative care is midazolam, which is a benzodiazepine prescribed in 9 of the 11 studies evaluated (Fig 2).3,14 Midazolam is prescribed in a wide dose range; for example, in 1 study, 61% of patients used doses no more than 30 mg and 8% of patients used doses of at least 120 mg.47 The highest final daily dose of midazolam was correlated with age (the younger the patient, the higher the dose) and treatment duration (the longer the treatment, the higher the dose).47 Midazolam has a rapid onset of action and short half-life, and both of these pharmacokinetic features facilitate the titration procedure during the first phase of sedation. When an adequate minimum dose (or, in some settings, a loading dose) has been identified for symptom control, a maintenance dose should be started by continuous intravenous or subcutaneous infusion. Because midazolam has anticonvulsant, muscle-relaxant, hypnotic, and anxiolytic properties, it can be added to other sedative classes to achieve control of these specific symptoms. Some studies advocate use of neuroleptics for delirium (eg, haloperidol).35-37 Haloperidol has less sedating power than midazolam, so it is typically used to attenuate delirium; thus, it is not the most appropriate choice of drug for achieving continuous sedation. Other drugs used for palliative sedation have included levomepromazine, chlorpromazine (especially when profound sedation is needed for acute agitated delirium), propofol, ketamine, and dronabinol.39,48-51 If a patient undergoing sedation is treated with an opioid for pain or dyspnea, then the opioid must not be interrupted. By contrast, opioids should not be used for sedation, because doing so would make use of their secondary effect (somnolence) and would be considered secondary palliative sedation, which is not recommended.3,5,8-10 Duration 436 Cancer Control 191 (26%) Chlorpromazine Drug The mean or median length of palliative sedation ranges from 0.8 to 12.6 days.12 One study observed an even larger range (0–43 days), despite mean and median times in line with the literature (4 and 2 days, respectively).13 In this study, 10.8% of patients underwent palliative sedation for more than 10 days and 3.4% of patients for more than 20 days.13 Patients requiring sedation for more than 10 days had fewer rates of delirium and dyspnea, milder and more frequent use of secondary palliative sedation, and higher rates of psychological distress than their counterparts.13 362 (49%) Midazolam Haloperidol 107 (14%) Morphine Methotrimeprazine Propofol 79 (11%) 22 (3%) 11 (1%) Phenobarbital Other benzodiazepines 5 (1%) 161 (22%) 0 100 200 300 400 Patients Fig 2. — Sedative drugs administered to 745 patients from 9 studies. From Maltoni M, Scarpi E, Rosati M, et al. Palliative sedation in end-of-life care and survival: a systematic review. J Clin Oncol. 2012;30(12):1378-1383. Reprinted with permission. © 2012. American Society of Clinical Oncology. All rights reserved. October 2015, Vol. 22, No. 4 Monitoring Guidelines for palliative sedation recommend close surveillance of patients with respect to the management and relief symptoms and suffering, depth of sedation (level of consciousness), and potential adverse events of sedation.3-10 Family members of patients and their health care team should be monitored for psychological and spiritual distress.3,5,6,8 Vital signs should be assessed in nonimminently dying patients undergoing short-term, intermittent, or light sedation. Evaluating symptom relief or relief of suffering in unconscious patients can be difficult. Some scores monitoring verbal or facial expression, body movements, and response to nonpainful stimuli may be useful when deep sedation is performed, and some authors have suggested using the level of sedation as a proxy for evaluating symptoms.3,25,31 Depth of sedation is a common theme in studies and the tools used to assess it can vary. For example, guidelines from the European Association for Palliative Care recommend the Critical-Care Pain Observation Tool or the Richmond Agitation-Sedation Scale (RASS).52,53 A prospective study performed by Arevalo et al54 evaluated the validity and reliability of 4 scales: the Minnesota Sedation Assessment Tool, the RASS, the Vancouver Interaction and Calmness Scale, and a sedation score proposed by the Royal Dutch Medical Association. The RASS was as reliable as the score proposed by the Royal Dutch Medical Association and study results claimed that the RASS was the least time consuming, clearest, and easiest to use of the 4 tools.54,55 Another study tested a modified form of RASS (RASS-PAL) for use in patients in the palliative care unit, observing that the tool was useful for assessing sedation; however, further validation studies are needed to confirm these results.56 More objective methods to evaluate depth of sedation have been proposed because some sedated patients may continue to experience symptoms without being able to communicate their distress.57,58 Deschepper57 proposed a mixed-evaluation method that combined use of a scale, a subjective assessment by professionals, as well as neuroimaging, electrophysiological techniques, or both. However, such an approach is impractical in end-of-life care and may be more suited to a research setting.57 A systematic review of the literature revealed that 5 clinical studies and 1 guideline-based article included use of a validated scale to evaluate the results of palliative sedation.58 During the titration phase of sedation, clinical parameters should be evaluated every 15 to 30 minutes, but the exact interval should be calculated on the basis of the drugs, doses used, and clinical conditions of the patient; once an appropriate level of sedation has been achieved, the frequency of assessment can be reduced to once every 24 hours.3 One Cochrane review evaluated the impact October 2015, Vol. 22, No. 4 of palliative sedation on quality of life and participant well-being.15 None of the 14 studies included in the review took any of the above issues into consideration.15 The impact on symptoms was partially reported and with different methods, thus making data pooling impossible.15 Furthermore, the studies reviewed had numerous biases, with the authors concluding that the data are insufficient and the evidence is of poor quality with regard to the qualitative effectiveness of palliative sedation.15 They also recommended that studies on palliative sedation focus on qualitative, rather than quantitative, end points (ie, length of survival from the start of palliative sedation).15 Impact on Survival For some, the impact of palliative sedation on survival is not an issue that merits much attention, given that the priority of palliative sedation in the end-of-life setting is quality of life; thus, even a detrimental effect on survival may not be considered of particular relevance.59 By contrast, some physicians may consider it fundamental to know whether the sedation proposed has a high, medium, or low risk of hastening the death of their patient.14 However, this question cannot be studied with the highest evidence-based methodology. Although the authors of the Cochrane review were unable to pool data on qualitative outcomes of palliative sedation, they did provide reasonably good, quantitative evidence that palliative sedation does not have a detrimental impact on survival.15 The authors looked at 14 studies involving 4,167 adults — nearly one-third of whom were sedated.15 Survival time measured from admission or referral to death was not statistically significant between those who were sedated and those who were not.15 A prospective study of patients admitted to hospices matched patients who were sedated and those who were not for sex, age, reason for admission, performance status, and prognostic score.13 Survival from admission to death was compared between the 2 groups and no detrimental effect of palliative sedation on survival was found.13 Of note, even intensive procedures, deep continuous sedation or continuous sedation until death, did not hasten death when performed in a proportional, step-by-step manner.13 A subsequent systematic review also identified 11 studies totalling approximately 2,000 patients.14 No difference in survival was seen between patients receiving hospice care who underwent sedation (median, 7–27 days) and those who did not (median, 4–40 days).14 Such results confirm that palliative sedation can be considered a legitimate clinical intervention from most ethical viewpoints and that the principle of double effect is unnecessary to justify its practice. This ethical criterion is appropriate in a small percentage of patients receiving sedation in whom respiratory or circulatory function depression is recorded following Cancer Control 437 palliative sedation (3%–4%) and hypothesized as having potentially hastened death.60 In that sense, it may be appropriate to consider this effect a serious adverse event, which could occur after any medical or surgical procedure. Ethical Considerations If a survival impact of palliative sedation was ever demonstrated, then palliative sedation might have been equated with “slow” or “soft” physician-assisted suicide; however, absence of a detrimental impact on survival is sufficient to distinguish palliative sedation from physician-assisted suicide. The European Association for Palliative Care has identified 3 areas in which palliative sedation differs from euthanasia11: • The intention of palliative sedation is to provide relief from unbearable suffering caused by a refractory symptom, whereas euthanasia is designed to end the life of someone who is suffering. • Palliative sedation is a proportionate and symptom intensity–guided procedure using the minimum useful dose of a sedative drug (eg, a benzodiazepine); it is individually oriented and monitored. Conversely, euthanasia uses neuromuscular relaxants and barbiturates and is not monitored on the basis of the symptom relief obtained.61,62 • Success of palliative sedation is measured in terms of the relief it provides from distress. Although palliative sedation does not play a role in hastening death, its use prevents many patients from maintaining verbal contact with their relatives, which makes it unique among medical interventions.63 Injudicious uses of palliative sedation include inadequate assessment of potentially reversible causes of severe symptoms, lack of involvement of specialists in the condition underlying the presenting symptoms, excessive use by overwhelmed health care professionals, performing the procedure at the request of the relatives, and untimely start (ie, too early, too late).6,12,60 Although some may view palliative sedation, deep continuous sedation, or continuous sedation until death as bringing patients to a sort of “living dead-like” state in which they are no longer considered persons, it is our opinion that patients who are sedated can be talked to, wanted, cared for, and loved by their relatives, sometimes “answering” with their presence in surprising and unexpected ways.22,28 The “innate” core of personhood (“individual component”) has been suggested to persist even after relational and societal components are severely reduced.29 Nutrition and Hydration Those who believe that palliative sedation plays a role in hastening death may place most of the responsibility for this “secondary effect” on the simultaneous in438 Cancer Control terruption of artificial nutrition and hydration — both of which are considered medically assisted treatments or as vital support interventions, at least in this patient setting. However, medically assisted hydration is a topic more often discussed, given that artificial nutrition may be interrupted prior to palliative sedation due to its proven lack of impact on the duration and quality of life of patients with a terminal disease.61-66 The role of hydration in end-of-life care is subject to different perspectives and is widely discussed in the literature.64 In certain situations, excess hydration can lead to water retention and exacerbation of pleural or peritoneal effusion, among other problems; by contrast, interrupting liquids may provoke thirst and an increase in drug metabolites, worsening delirium, and agitation, thus making it difficult for patients to communicate distress.65 Thus, maintenance or withdrawal of hydration must be individually evaluated and managed. Results from a large epidemiological study on 20,480 questionnaires completed by physicians experienced in deep continuous sedation or continuous sedation until death showed that, even in this “extreme” form of sedation, up to two-thirds of patients continued hydration for clinical reasons.66 The take-home message is that use of palliative sedation and evaluation of whether or not to continue hydration involve 2 different decision-making processes. Thus, hydration does not have to be automatically stopped because palliative sedation has begun. Communication and Decision-Making Process Talking about death with patients and their families is a delicate and challenging aspect of palliative care. Ideally, discussions of this kind should be initiated when a patient’s prognosis is years to months and then reevaluated when the patient’s life expectancy decreases to months to weeks.9 The conversation should be a balance between maintaining hope and realistic and achievable goals.6,67 Health care professionals must plan these end-of-life interventions by taking into account the goals, values, needs, and wishes of the patient. Patients should be asked where they prefer to die so that adequate plans can be made to respect their request. All decisions should be documented in the patient’s medical records.9 When a health care professional recognizes the imminence of suffering, he or she should discuss the possibility of sedation with the patient, outlining the aims, risks, and benefits of the procedure. The patient may not want to broach the subject and may authorize the health care professional to make decisions on his or her behalf or delegate a caregiver to do so. If the patient is no longer capable of making an autonomous decision, then the health care professional may approach a family member or a surrogate decision maker to clarify the patient’s wishes.6 October 2015, Vol. 22, No. 4 A multidisciplinary team of oncologists, palliative care specialists, psychologists, psychiatrists, nurses, chaplains, or spiritual advisors, among others, may also form the decision-making group, together with the patient and his or her family.5 In particular, such a team can help ensure that all conditions have been met for palliative sedation, that informed consent is obtained, that the type of intervention has been planned, and then modify any plans, when and if necessary, during the course of sedation. Further therapeutic interventions may also be performed if other conditions arise (eg, urinary retention, constipation, myoclonus, “death rattle”). Keeping an open dialog with relatives and the patient’s loved ones throughout the palliative sedation process is fundamental so that they understand the characteristics of this medical intervention (ie, it is performed in the absence of other means to manage refractory and unbearable symptoms; it neither hastens nor postpones death; it reduces or eliminates the possibility of verbal communication with patients; clinical conditions permitting, it is reversible in nature; it is carefully monitored to avoid oversedation or undersedation; it is performed for the well-being of patients).68,69 Although some relatives experience psychological distress, Bruinsma et al69 reported that most are comfortable with palliative sedation if they are appropriately informed about the procedure and actively involved in the decision-making process. A study of Swiss caregivers of patients who died while undergoing palliative sedation found that most caregivers agreed about the timing of the initiation of palliative sedation and confirmed that it led to a substantial improvement in refractory symptoms.70 In a small study of Dutch relatives of patients receiving palliative sedation until death, many of the relatives acknowledged that palliative sedation was indicated, but they felt that communication between the health care professionals and the relatives was poor.71 Although an open dialog between the health care team, the patient, and his or her family members and loved ones is important, we do not feel that such open communication is always respected. In an Italian study of communicative behavior in 2 different hospice settings, family involvement in the decision-making process with regard to palliative sedation was 100% in both hospices, but the rate of patient participation in such decision-making varied from 24% to 59%.35 Another study of Dutch and US physicians revealed that open discussion among physicians, patients, and families was more diffuse in The Netherlands than in the United States.72 However, the study involved too few cases to conclude that an international difference exists when communication about sedation due to different cultural approaches.72 In a group of 27 sedated October 2015, Vol. 22, No. 4 patients, a Portuguese case study determined that the decision to begin sedation was made by health care professionals alone in 21 cases.38 The authors attributed this result to the fact that 76% of study patients required urgent and nonpostponable sedation.38 Nurses also play an essential role in the decisionmaking process. A survey of 576 nurses from The Netherlands showed that most responders had cared for at least 1 patient who received palliative sedation.73 Nearly all of the nurses indicated that they had been involved in the decision to perform palliative sedation and were present when palliative sedation was started, unlike the physicians who were present in fewer than 50% of cases.73 A study of Flemish nurses also revealed that the majority of the nurses supported use of palliative sedation for refractory symptoms.74 Patel et al75 studied the attitudes and perspectives of nurses working with patients receiving palliative sedation in 3 different settings (oncology, intensive care, and hospice), highlighting their ability to define palliative sedation, their skill set for administering palliative sedation, policy, and procedural guidelines, and their education on palliative sedation and end-of-life care. Guidelines Many clinical associations and international agencies have produced procedural guidelines for use of palliative sedation so as to provide health care professionals with a framework on which to base the clinical decision-making process.3-10 Key issues in palliative sedation were also addressed in a 10-item framework created by the European Association for Palliative Care (Table 2).5,6 An audit performed in a single center to assess adherence to guidelines on palliative sedation in Table 2. — European Association for Palliative Care 10-Item Framework for Guidelines in Palliative Sedation Need for preemptive discussion of potential role of sedation in end-of-life care and contingency planning Description of when sedation is indicated Description of necessary evaluation and consultation procedures Indications for informed consent requirements Indications for need to discuss decision-making process with patient’s family Indications for selecting method of sedation Indications for dose titration, patient monitoring, and care Indications for decisions regarding hydration, nutrition, and concomitant medications Indications for needs of the patient’s family Support for medical and nursing staff From Cherny NI; ESMO Guidelines Working Group. ESMO clinical practice guidelines for the management of refractory symptoms at the end of life and the use of palliative sedation. Ann Oncol. 2014;25(suppl 3):iii143-11152. Reprinted by permission of Oxford University Press. Cancer Control 439 a palliative care unit confirmed an adherence rate of 100%.76 Conversely, a survey of Dutch general practitioners revealed a high resistance to following guidelines, and this was particularly true with regard to the inadvisability of using palliative sedation without previous expert consultation and to the recommendation of the involvement of a multidisciplinary palliative care team.5,77 Without such an approach, misconceptions about palliative sedation may be further misconstrued and perpetuated. Conclusions Some patients with cancer experience refractory symptoms during the last hours or days of their life and are no longer responsive to antisymptomatic treatments. Managing such symptoms is the objective of palliative sedation, a proportional medical approach that must be individually tailored to each patient and closely monitored.3-10 Undersedation and oversedation can be avoided by titrating the level of sedating drugs to the minimum useful dose (termed proportionate palliative sedation). A different approach involving standardized drug dosages and deep continuous sedation or continuous sedation until death risks being performed with the total or partial intention to hasten death.3-10 However, palliative sedation should only be used in a proportional way; such a view has been accepted by many bioethical viewpoints. Although evidence suggests that proportionate palliative sedation does not have a detrimental impact on survival, further research is needed to monitor the qualitative results of the procedure.14,15 Benzodiazepines — in particular, midazolam — are the first choice of drugs for this type of sedation. The decision-making process to initiate proportionate palliative sedation is complex and should involve patients, their relatives and loved ones, and the health care team. This process varies from those used for other clinical decisions such as maintenance or withdrawal of artificial nutrition and hydration. Proportionate palliative sedation is a universally accepted medical intervention that differs from physician-assisted suicide in terms of intention, procedure, and results. Acknowledgments: The authors thank Gráinne Tierney and Ursula Elbling for editorial assistance. References 1. Hasselaar JG, Verhagen SC, Vissers KC. When cancer symptoms cannot be controlled: the role of palliative sedation. Curr Opin Support Palliat Care. 2009;3(1):14-23. 2. De Graeff A, Dean M. Palliative sedation therapy in the last weeks of life: a literature review and recommendations for standards. J Palliat Med. 2007;10(1):67-85. 3. Schildmann EK, Schildmann J, Kiesewetter I. 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Efficacy and safety of palliative sedation therapy: a multicenter, prospective, observational study conducted on specialized palliative care units in Japan. J Pain Symptom Manage. 2005;30(4):320-328. 61. Rietjens JA, van Delden JJ, van der Heide A, et al. Terminal sedation and euthanasia: a comparison of clinical practices. Arch Intern Med. 2006;166(7):749-753. 62. Hahn MP. Review of palliative sedation and its distinction from October 2015, Vol. 22, No. 4 euthanasia and lethal injection. J Pain Palliat Care Pharmacother. 2012;26(1):30-39. 63. Janssens R, van Delden JJ, Widdershoven GA. Palliative sedation: not just normal medical practice. Ethical reflections on the Royal Dutch Medical Association’s guideline on palliative sedation. J Med Ethics. 2012;38(11):664-668. 64. Bruera E, Hui D, Dalal S, et al. Parenteral hydration in patients with advanced cancer: a multicenter, double-blind, placebo-controlled randomized trial. J Clin Oncol. 2013;31(1):111-118. 65. Beland P. Ethical issues around continuous deep sedation without hydration. Nurs Times. 2012;108(38):22-25. 66. Miccinesi G, Rietjens JA, Deliens L, et al. Continuous deep sedation: physicians’ experiences in six European countries. J Pain Symptom Manage. 2006;31(2):122-129. 67. Harrison JD, Young JM, Price MA, et al. What are the unmet supportive care needs of people with cancer? A systematic review. Support Care Cancer. 2009;17(8):1117-1128. 68. Maltoni M, Scarpi E, Nanni O. Palliative sedation in end-of-life care. Curr Opin Oncol. 2013;25(4):360-367. 69. Bruinsma SM, Rietjens JA, Seymour JE, et al. The experiences of relatives with the practice of palliative sedation: a systematic review. J Pain Symptom Manage. 2012;44(3):431-445. 70. Vayne-Bossert P, Zulian GB. Palliative sedation: from the family perspective. Am J Hosp Palliat Care. 2013;30(8):786-790. 71. Bruinsma S, RietjensJ van der Heide A. Palliative sedation: a focus group study on the experiences of relatives. J Palliat Med. 2013;16(4):1-7. 72. Rietjens JA, Voorhees JR, van der Heide A, et al. Approaches to suffering at the end of life: the use of sedation in the USA and Netherlands. J Med Ethics. 2014;40(4):235-240. 73. Arevalo JJ, Rietjens JA, Swart SJ, et al. Day-to-day care in palliative sedation: survey of nurses’ experiences with decision-making and performance. Int J Nurs Stud. 2013;50(5):613-621. 74. Gielen J, Van den Branden S, et al. Flemish palliative-care nurses’ attitudes to palliative sedation: a quantitative study. Nurs Ethics. 2012;19(5):692-704. 75. Patel B, Gorawara-Bhat R, Levine S, et al. Nurses’ attitudes and experiences surrounding palliative sedation: components for developing policy for nursing professionals. J Palliat Med. 2012;15(4):432-437. 76. Benitez-Rosario MA, Castillo-Padrós M, Garrido-Bernet B, et al. Quality of care in palliative sedation: audit and compliance monitoring of a clinical protocol. J Pain Symptom Manage. 2012;44(4):532-541. 77. Koper I, van der Heide A, Janssens R, et al. Consultation with specialist palliative care services in palliative sedation: considerations of Dutch physicians. Support Care Cancer. 2014;22(1):225-231. Cancer Control 441 Progress has been made in recent years for integrating psychosocial care into routine cancer care, but more work is needed. Photo courtesy of Lisa Scholder. Seizing the Light. 16" × 20". Integrating Psychosocial Care Into Routine Cancer Care Paul B. Jacobsen, PhD, and Morgan Lee, MA Background: Despite growing recognition that psychosocial care is an essential component of comprehensive cancer care, evidence suggests many patients with cancer do not receive needed psychosocial care. Methods: Four areas were identified as potentially increasing the number of patients with cancer who receive needed psychosocial care: (1) formulating care standards, (2) issuing clinical practice guidelines, (3) developing and using measurable indicators of quality of care, and (4) demonstrating projects designed to improve the delivery of care. Results: Standards for psychosocial care are identified, including a standard issued in 2015 by an accrediting organization. Three clinical practice guidelines for provisioning psychosocial care are also identified and reviewed. Methods for monitoring the quality of psychosocial care are characterized and the impact of monitoring changes in quality are evaluated in relation to existing evidence. Examples are provided of 2 large-scale efforts designed to improve the delivery of psychosocial care in community settings. Conclusions: Although considerable progress has been made in integrating psychosocial care into routine cancer care, work must still be done. Additional progress will be fostered by continued efforts to promote adherence to clinical practice guidelines and care standards for psychosocial care and by the development and dissemination of models that demonstrate how practices can implement these guidelines and standards. Introduction Recognition is growing that psychosocial care is an essential component of the comprehensive care of people diagnosed with cancer.1,2 In addition to attempting to extend survival rates in people following a cancer diagnosis, the oncology community is recognizing the value of quality of life. Psychosocial care, with its goals of relieving emotional distress and promoting wellFrom the Department of Health Outcomes and Behavior (PBJ), H. Lee Moffitt Cancer Center & Research Institute, and the Department of Psychology (ML), University of South Florida, Tampa, Florida. Submitted April 8, 2015; accepted July 15, 2015. Address correspondence to Paul B. Jacobsen, PhD, 12902 Magnolia Drive, Tampa, FL 33612. E-mail: Paul.Jacobsen@Moffitt.org No significant relationships exist between the authors and the companies/organizations whose products or services may be referenced in this article. 442 Cancer Control being, is central to efforts to improve quality of life.2 However, evidence suggests that many patients with cancer who might benefit from psychosocial care do not receive it.2 Four areas of activity have the potential to increase the number of patients who receive needed psychosocial care: 1. Formulating care standards that address the psychosocial component of care 2. Issuing clinical practice guidelines for the psychosocial care of patients with cancer 3. Developing and using measurable indicators of quality of psychosocial care in oncology settings 4. Demonstrating projects designed to promote the greater implementation of standards for psychosocial care October 2015, Vol. 22, No. 4 Definitions Standards in medical care refer to diagnostic or treatment processes that health care professionals should follow for certain classes of patients, illnesses, or clinical circumstances. Standards may be developed based on evidence, expert consensus, and/or ethical and safety considerations. With regard to the psychosocial domain, standards of care represent recommendations for the organization and delivery of psychosocial care that apply to patients seen in the oncology setting. Clinical practice guidelines are systematically developed statements designed to assist health care professionals and patients in making decisions about appropriate health care based on specific characteristics of the patient (eg, age, comorbidities), illness (eg, disease severity), or clinical circumstances (eg, symptom presentation). Similar to standards, clinical practice guidelines can be developed based on evidence, expert consensus, and/or ethical and safety considerations. In general, measuring the quality of care involves assessing the extent to which an organization and delivery of care conforms to standards of care and clinical practice guidelines. A widely used model dating from the 1960s differentiates 3 components important to consider in evaluating quality, namely: (1) the structure of care (eg, resources or personnel), (2) the processes of care (eg, performance of specific diagnostic procedures or treatments), and (3) outcomes of care (eg, survival rates).3 Methods for assessing the quality of psychosocial care have primarily focused on evaluating processes of care. Table. — Select Standards for Psychosocial Care of Patients With Cancer Professional Group American College of Surgeons Commission on Cancer 5 Specifies standards for organizing, delivering, and monitoring of oncology services Presented as requirements evaluated during accreditation review Key example of standards for psychosocial care: • Cancer committee develops and implements a process to integrate and monitor on-site psychosocial distress screening and referral for psychosocial care Canadian Association of Psychosocial Oncology4 Specifies standards of care for organizing and delivering psychosocial health services Presented in sections (key principles, organization and structure, educational standards for providers, standards of care) Key examples of standards for psychosocial care: • People at risk for or living with cancer are entitled to psychosocial screening using a standardized approach • People affected by cancer are entitled to access appropriate levels of treatment to address their needs Institute of Medicine2 Specifies standards for providing appropriate psychosocial services Presented as processes and goals of care Key examples of processes and goals of psychosocial care: • Facilitate effective communication between patients and health care professionals • Identify psychosocial health needs of each patient • Design and implement a plan to link patient with needed services • Follow-up on, re-evaluate, and adjust plan National Comprehensive Cancer Network 6 Specifies standards of care for distress management Presented as imperatives focused on distress management Key examples of standards for distress management: • Distress should be recognized, monitored, documented, and promptly treated • Screening should identify the level and nature of distress • Distress should be assessed and managed according to clinical practice guidelines • Interdisciplinary institutional committees should be formed to implement standards for distress management • Experienced licensed mental health professionals and certified chaplains should be available as staff members or by referral Standards Efforts to promote greater awareness of the importance of psychosocial care for patients with cancer received a boost following a 2008 publication from the Institute of Medicine (IOM) summarizing evidence regarding the deleterious effects of unmet psychosocial needs and benefits of providing psychosocial services.2 Despite evidence supporting the effectiveness of psychosocial services, the IOM concluded that many patients do not receive help for problems that might benefit from this type of care.2 To address this problem, the report included a list of recommended actions, including that all entities establishing or using standards for the quality of cancer care adopt a standard that calls for the provision of appropriate psychosocial health services.2 The recommendation further identifies certain processes and goals of care as being components of this standard (Table).2,4-6 Several initiatives predate the IOM report in proposing standards that address psychosocial care. For example, the National Comprehensive Cancer Network (NCCN) included standards of care as part of its clinical practice guidelines for the management of cancerOctober 2015, Vol. 22, No. 4 Summary and Comments related distress first published in 1999.7 In addition to identifying policies and procedures related to screening for and managing distress, the NCCN standards call for the formation of interdisciplinary committees Cancer Control 443 at each institution to implement guidelines for distress management and for the availability of trained, on-site professionals or by referral to deliver psychosocial care (see Table).2,4-6 Initiatives promoting standards for the psychosocial care of patients with cancer are not limited to the United States. For example, the Canadian Association of Psychosocial Oncology developed standards in 1999 and updated them again in 2010.4,8 In addition to identifying procedures for psychosocial screening and treatment, these standards cover the organization and structure of psychosocial services and the education and training of psychosocial care providers.4 Most standards for psychosocial care are developed by organizations and committees composed of members of the psychosocial oncology clinical and research communities. Thus, concerns exist about the extent to which the wider oncology community has been cognizant of these standards and has adopted them.9 Efforts to promote the adoption of such standards would benefit patients if major accrediting organizations included psychosocial care among their standards. One such organization is the American College of Surgeons (ACS) Commission on Cancer (CoC), which is a consortium of 47 professional organizations.­10 The ACS CoC establishes cancer care standards and monitors the quality of care at approximately 1,500 hospitals, which are estimated to provide care to 70% of patients with cancer in the United States.10 In 2012, the ACS CoC released several standards for patientcentered care.5 Among them is a standard specifying that a local oversight committee should develop and implement a process for psychosocial distress screening and referral for psychosocial care (see Table).2,4-6 These standards are being evaluated in 2015 as part of the ACS CoC accreditation process.11 Clinical Practice Guidelines Worldwide, numerous organizations have proposed clinical practice guidelines that include recommendations for the psychosocial care of people with cancer.12 For brevity, only the details of 3 North American– based guidelines for psychosocial care will be covered in this article. National Comprehensive Cancer Network Clinical practice guidelines from the NCCN for distress management were first issued in 19997; they are updated every year and include recommendations for psychosocial screening, evaluation, treatment, and followup primarily presented in the form of algorithms or decision pathways.6 Most of the recommendations represent uniform consensus among experts from NCCN member institutions based on lower-level evidence (eg, clinical experience of expert providers) rather than 444 Cancer Control higher-level evidence (eg, results of randomized controlled trials). Recommendations for the management of mood disorders (eg, major depression) help illustrate how the clinical practice guidelines are organized. For example, the NCCN guidelines recommend that all patients undergo brief psychosocial screening for distress using a valid and reliable self-report tool.6 The importance of systematic screening is underscored by research indicating that oncologists typically underestimate the level of distress in their patients.13-15 For patients who have moderate to severe distress, referral to psychosocial care professionals is recommended.6 If patients are displaying signs and symptoms of a mood disorder, the initial recommendation is further evaluation, diagnostic studies, and modification of the factors potentially contributing to the symptoms (eg, concurrent medications, pain).6 Based on these findings, subsequent recommendations may include initiating psychotherapy and antidepressant medication, possibly in combination with anxiolytic medication. Consideration of referral to social work or chaplaincy services is also recommended before follow-up and re-evaluation.6 Pan-Canadian In 2010, the Canadian Partnership Against Cancer and the Canadian Association of Psychosocial Oncology jointly issued the Pan-Canadian guidelines based on methodology developed by the ADAPTE Collaboration.16 Development of the guidelines began with a systematic search to identify other relevant guidelines, systematic reviews, and guidance documents — a process that led to the formulation of a guideline presented in the form of recommendations (accompanied by information on the level of supporting evidence) and an algorithm describing the process for screening, assessing, and managing depression and anxiety.16 Unlike the NCCN guidelines that address a wide range of psychiatric disorders and psychosocial problems, the Pan-Canadian guidelines focus on depression and anxiety.6,16 Using depression as an example, the guidelines include specific recommendations for screening, assessing, and treating depression.16 These recommendations are similar in many respects to those in the NCCN guidelines, in part because the NCCN guidelines were part of the systematic search during the creation of the Pan-Canadian guidelines.16 However, the algorithm does differs from the NCCN algorithm; for example, the Pan-Canadian algorithm recommends screening for depression rather than distress, and it identifies separate care pathways based on the severity of depression rather than on the type of mood disorder.16 American Society of Clinical Oncology The American Society of Clinical Oncology (ASCO) October 2015, Vol. 22, No. 4 issued clinical practice guidelines in 2014 for the screening, assessment, and care of anxiety and depressive symptoms in adults with cancer.17 The ASCO guidelines were adapted from the Pan-Canadian guidelines, so they also used ADAPTE methodology and many of the ASCO recommendations and algorithms mirror those in the Pan-Canadian guidelines.17 However, the ASCO panel modified the Pan-Canadian guidelines in several instances and developed new recommendations based on additional evidence and expert opinion.17 Using the recommendations on depression as an example, the ASCO guidelines specify distinct care pathways based on the severity of depression.17 By contrast to the Pan-Canadian guidelines, which recommend use of the Edmonton Symptom Assessment System to screen for depression, the ASCO guidelines recommend the Patient Health Questionnaire.16,17 In addition, the ASCO guidelines include detailed recommendations for follow-up of patients identified as having depression.17 Guideline Harmonization Having 3 or more clinical practice guidelines from Canada and the United States, along with numerous other guidelines from other countries, may cause confusion among health care professionals and patients with cancer seeking guidance about when and how psychosocial care should be delivered.12 A possible solution to this problem is a process known as guideline harmonization. One such example of guideline harmonization is the ongoing, worldwide collaboration designed to standardize various clinical practice guidelines for the long-term follow-up of children and young adults with cancer; this collaboration has resulted in harmonized guidelines for breast cancer surveillance in women with cancer who have received chest irradiation.18,19 Similar to this approach, relevant stakeholders should seek to develop a set of harmonized guidelines that address the screening, assessment, and management of more common psychosocial problems encountered by people with cancer, beginning with depression and anxiety. Measuring Quality A paucity of data exists about the extent to which standards and clinical practice guidelines for psychosocial care of patients with cancer are being implemented. One source of evidence is a survey of 20 NCCN member institutions completed by a representative of each institution.20 Although psychosocial services were available at 19 of these institutions, only 12 institutions (60%) were conducting routine outpatient screening for distress, as stipulated in the NCCN standards of care and clinical practice guidelines for distress management.6,20 Among these 12 institutions, 6 reported October 2015, Vol. 22, No. 4 routinely screening all outpatients as recommended and 6 reported routinely screening select patients (eg, candidates for transplant).20 Of the 14 institutions conducting any routine inpatient or outpatient screening, 13 (93%) reported that, consistent with NCCN guidelines, patients identified as being in distress were referred to a mental health professional.6,20 The IOM report and the findings of an NCCN survey suggest the need to foster greater implementation of recommendations for the psychosocial care of patients with cancer.2,20 One way to foster greater implementation might be to measure and provide feedback to health care professionals about the quality of the psychosocial care their patients with cancer receive. Research has shown that medical oncology practices provided with feedback demonstrating their poor performance on quality indicators will improve over time on those same indicators.21 Could psychosocial care for patients with cancer likewise be improved by measuring and reporting to oncology practices their performance on indicators of the quality of psychosocial care? To help answer this question, the IOM recommended that organizations setting standards for cancer care use performance measures for psychosocial care as part of quality-oversight activities.2 The first step in this process is to develop measurable indicators of the quality of psychosocial care. Toward this end, the American Psychosocial Oncology Society formed a workgroup in 2007 charged with developing quality indicators.22 Members of the workgroup included 5 mental health professionals (psychologists, psychiatrists, and social workers) with extensive experience in the delivery of psychosocial care to patients with cancer. The committee focused on developing process measures of the quality of psychosocial care that could be evaluated by medical record abstraction.22 Following a review of the relevant literature, including the IOM report and the NCCN guidelines, committee members identified several potential indicators that were then reduced in number using a modified Delphi method.2,6,22 This process resulted in selection of measures assessing 2 components considered to be necessary (although not sufficient) for providing quality psychosocial care.22 The first quality indicator specifies evidence should exist in the patient’s medical record that his or her current emotional well-being was assessed within 1 month of the patient’s first visit with a medical oncologist.22 The second quality indicator stipulates that, if a problem with emotional well-being was identified, then evidence should exist in the patient’s medical record supporting that action was taken to address the problem or an explanation provided for why no action was taken.2 Measuring these indicators is operationalized by formulating questions that can be answered “yes” or “no” based on the review of an individual paCancer Control 445 tient’s medical record.22 Two sources offer preliminary evidence that suggests providing feedback on the quality of psychosocial care might lead to improvements in care. One source is ASCO’s Quality Oncology Practice Initiative, a voluntary, practice-based quality improvement program.23 In 2008, 2 quality indicators for psychosocial care were added to its core set of measures completed by all participating practices.24 Practices participating in the practice initiative have the opportunity to submit chart audit information at 6-month intervals.23 Following submission of their data, practices are given performance feedback on each indicator as well as the average performance score of all other participating practices.23 Using data provided by participating practices, an analysis was conducted to determine whether performance on these indicators improved between fall 2008 (when they became part of the core set) and fall 2009.25 The analysis was based on 166 participating practices and data from approximately 15,000 patients at each time point.25 The average rate per practice for performing an assessment of emotional well-being improved over time, from 64% to 73% (P < .001).25 By contrast, the average rate per practice for taking action if a problem with emotional well-being was identified increased from 74% to 76% (P = .41).25 Additional evidence comes from the Florida Initiative for Quality Cancer Care.26 As part of a larger project examining quality of cancer care, performance rates for the 2 psychosocial indicators were available for 10 practice sites in Florida that completed chart audits of patients with colorectal, breast, or non–smallcell lung first seen by a medical oncologist in 2006 (n = 1,609) and 2009 (n = 1,720).26 Following the 2006 chart audit, all 10 practices received feedback on their performance and were encouraged to develop their own quality improvement efforts if performance rates were below 85%.26 The mean percentages of patients whose emotional well-being was assessed were 53.1% in 2006 and 51.3% in 2009, reflecting a nonsignificant decrease (P = .661).26 However, significant increases were seen in the prevalence of documented problems in emotional well-being among all patients (from 13.0% to 16.0%) and among patients whose emotional well-being was assessed (from 24.5% to 31.3%; P ≤ .021).26 The percentages of patients for whom action was taken to address a problem in emotional wellbeing were 57.4% in 2006 and 45.3% in 2009, thus reflecting a nonsignificant decrease (P = .098).26 Taken together, these findings suggest that providing feedback alone may be more effective in promoting psychosocial screening, identifying distressed patients, or both, than improving the delivery of psychosocial care to patients in need.23,25,26 Efforts to improve the delivery of psychosocial care to patients in distress are likely to face a number of additional barriers, includ446 Cancer Control ing competing clinical priorities, poor reimbursement for mental health services, and lack of psychosocial staff to accept referrals.9,27 This situation points to the need to conduct demonstration projects that seek to identify and test different approaches to improving the quality of psychosocial care in oncology settings. Consistent with this view, the IOM included a recommendation that federal funding agencies support a largescale demonstration and evaluation of how standards for psychosocial care could be implemented across diverse treatment settings.2 Demonstrating Approaches to Improving Care Several examples have been published of efforts to implement routine distress screening programs in oncology settings.28-31 These efforts vary widely in the tools used to screen, the methods used to collect information from patients and deliver it to clinicians, and whether the information obtained from patients is present to clinicians with referral recommendations — all of which are features that limit the ability to draw conclusions from this literature. However, reason exists to question the value of solely implementing distress screening without also implementing referral recommendations for distressed patients. Evidence for this concern can be found in a study that compared a usual practice condition in which oncologists rated their patients’ distress and decided whether referrals were indicated vs a screening condition in which oncologists received information about whether a patient’s level of distress exceeded a cut-off, thus suggesting referral to psychosocial care.32 Findings showed that 5.5% of patients in distress receiving standard care and 69.1% of patients in distress in the screening condition received referrals; in addition, 3.7% of patients in distress receiving standard care compared with 27.6% of patients in distress in the screening condition accepted the referral.32 Two projects illustrate larger multicenter efforts aimed at improving the psychosocial care of people with cancer. In 2007, the National Cancer Institute Community Cancer Centers Program (NCCCP) was initiated to enhance cancer research and the quality of cancer care in community hospitals.33 Influenced by IOM recommendations, the NCCCP working group developed a tool, the Cancer Psychosocial Care Matrix (CPCM), that defined 10 components of care comprising a comprehensive psychosocial care program.2,33 Within each component, the CPCM delineated measurable milestones designed to guide program development toward a site’s full potential for delivering psychosocial care.33 Specifically, the site is asked to identify which performance level (≤ 5 levels) they are demonstrating to deliver quality psychosocial care.33 For example, the CPCM includes an item that allows sites to conduct a self-assessment of the extent to which processes are put in place for meeting the IOM report October 2015, Vol. 22, No. 4 recommendation that psychosocial needs of patients be identified.33 A level 1 rating reflects no systematic screening process in place, and a level 5 rating reflects consistent systematic screening on multiple occasions from diagnosis through follow-up, accompanied by a comprehensive assessment for patients who screen positive.33 It should be noted that the NCCCP developed a similar tool for evaluating and improving palliative care services.34 Results suggest the CPCM was useful in evaluating the progress NCCCP sites had made in their goal to improve the quality of psychosocial care provided to their patients.33 In 2010, 16 NCCCP sites used the CPCM to provide retrospective ratings of their psychosocial program characteristics upon entry into the NCCCP as well as current ratings approximately 2 years later.33 Findings indicated that most of the baseline responses (60%) of the sites reflected level 1 responses (ie, lowest possible level of service delivery).33 Two years later, the majority of responses (59.4%) reflected level 2 to 4 responses (ie, intermediate levels of service delivery).33 In addition to quantifying progress in improving care, anecdotal findings indicated that the CPCM served at most sites to promote intentions to improve psychosocial services and that the ordered response options facilitated incremental growth toward a desired practice.33 The other example is a project that evaluated the feasibility of a quality improvement strategy for integrating psychosocial care at 27 medical centers in Italy.35 The strategy relied on context analysis and problem solving to facilitate implementation and involved 4 to 6 visits conducted in each center by the project team to assist clinic staff in identifying obstacles, finding solutions, and strengthening motivation to carry out recommended changes. Following an implementation period, the authors assessed adherence to each of the 6 recommendations and considered the objective to be met if the center’s adherence percentage was at least 75%.35 Implementation was generally successful, as indicated by the relatively few centers with adherence rates that fell below this criterion for each of the following 6 recommendations: clinician participation in communication skills training (1 center), provision of a question prompt list to each patient (7 centers), assignment of a specialist nurse to each patient (2 centers), completion of at least 1 psychosocial distress screening for each patient (3 centers), completion of at least 1 social need screening for each patient (3 centers), and an offer to visit an information and support center for each patient (3 centers).35 Although these results are promising, the participating medical centers were primarily leading centers of excellence, the sustainability of these outcomes was not assessed, and the evidence of improvement was limited to process indicators and not outcome indicators of quality (eg, patient psychological well-being).35 October 2015, Vol. 22, No. 4 Future Directions In retrospect, the IOM report can be seen as a turning point in the efforts to promote the integration of psychosocial care into routine cancer care.2 Although the IOM’s report was useful in summarizing the benefits of addressing psychosocial needs and the liabilities of not addressing them, its major impact has been to draw attention to the fact that many patients who might benefit from psychosocial care are not receiving it.2 In addition to focusing attention on the problem, the report included a number of recommendations that have served as an effective action plan for efforts to address the problem.2 Among the IOM report’s most important recommendations was one stipulating that entities establishing or using quality standards in oncology should include a provision of appropriate psychosocial health services among their standards.2 Although clinical practice guidelines for psychosocial care have been available since 1999, many reasons exist to believe that developing and disseminating guidelines are necessary steps but are insufficient when it comes to changing clinical practice.7 The development of standards of care is also required but experience suggests that, for this approach to be successful, the issuance of standards must move beyond initiatives developed and directed primarily by members of professional societies. A critical milestone occurred in 2012 when the ACS CoC issued standards requiring the development and implementation of processes for psychosocial distress screening and referrals for psychosocial care.5 Adherence to these standards is being evaluated in 2015 as part of the ACS CoC accreditation, so considerable motivation exists for many oncology care sites to evaluate and, if needed, improve their processes in this area.10 In anticipation of this new standard taking effect, several major professional societies involved in psychosocial care have collaborated to issue recommendations that address the 6 components of the standard36: 1. Overall plan for screening 2. Timing of screening 3. Method and mode of screening 4. Tools for screening 5. Assessment and referral 6. Documentation of screening and related actions in the medical record These recommendations build on published clinical practice guidelines.6 Another important recommendation that came out of the IOM report stipulated that the organizations setting standards for cancer care should implement performance measures for psychosocial care as part of quality oversight activities.2 The Quality Oncology Practice Initiative of ASCO is one of the largest cancerrelated quality monitoring systems in the United States, Cancer Control 447 with more than 900 registered practices.37 Spurred in part by the IOM report, the initiative adopted indicators of the quality of psychosocial care for its core module that are required of all participating practices.2,38 Findings based on audits of the Quality Oncology Practice Initiative and the Florida Initiative for Quality Cancer Care suggest that providing feedback about the quality of psychosocial care can have a positive impact on rates of psychosocial screening.25,26 However, change is lacking for taking action in cases where problems in emotional well-being were identified; thus, this finding suggests feedback alone is insufficient for improving the delivery of psychosocial services.25,26 Possible explanations for such findings include lack of referral procedures for psychosocial care, lack of identified resources for providing psychosocial care, or both. Oncology practices seeking to address these issues would benefit from knowing how other practices have responded to similar challenges. One source of information might be case studies describing how practices improved their provision of psychosocial care. An example of this type can be found in a publication that provided a description of how a regional cancer center developed counseling services to address the unmet psychosocial needs of its patients.39 The IOM report also recommended that funding agencies support a large-scale demonstration and evaluation of how standards for psychosocial care can be implemented across diverse treatment settings.2 This recommendation was addressed as part of the federally funded NCCCP. Based on guidance provided by the IOM, participating sites implemented planning efforts that resulted in substantial improvements in psychosocial care delivery.2,33 Additional reports have provided more in-depth descriptions of efforts to implement distress screening and psychosocial referral and the acceptability and impact on processes of care in those efforts.28-32,35 Work in this area would also benefit from published findings from rigorously designed, quality improvement projects designed to document the processes used to improve psychosocial care and the outcomes achieved, including the impact on patient quality of life. Such studies should also consider relevant conceptual frameworks such as the PRECEDE-PROCEED model, which focuses on identifying and influencing predisposing, enabling, and reinforcing factors for implementing changes.40,41 Reports of this type have yet to appear in the literature and should be considered a high priority for future efforts to promote psychosocial care for patients with cancer. Conclusions Although considerable progress has been made in recent years in integrating psychosocial care into routine 448 Cancer Control cancer care, much work remains to be done. Additional progress will be fostered by continued efforts to promote adherence to clinical practices guidelines and care standards for psychosocial services and through the development and dissemination of models demonstrating how practices can effectively implement these guidelines and standards. References 1. Hewitt M, Herdman R, Holland, eds; National Cancer Policy Board; Institute of Medicine; National Research Council. Meeting Psychosocial Needs of Women With Breast Cancer. Washington, DC: National Academies Press; 2004. 2. Adler NE, Page AE, eds; Institute of Medicine Committee on Psychosocial Services to Cancer Patients/Families in a Community Setting. Cancer Care for the Whole Patient: Meeting Psychosocial Health Need. Washington, DC: National Academies Press; 2008. 3. Donabedian A. Evaluating the quality of medical care. Milibank Mem Fund Q. 1966;44(3 suppl):166-206. 4. Canadian Association of Psychosocial Oncology (CAPO). Standards of Psychosocial Health Services for Persons with Cancer and their Families. Toronto, ON, Canada: CAPO; 2010. http://capo.ca/CAPOstandards.pdf. Accessed September 1, 2015. 5. Commission on Cancer. Cancer Program Standards 2012: Ensuring Patient-Centered Care. V1.2.1. Chicago: American College of Surgeons; 2012. https://www.facs.org/~/media/files/quality%20programs/cancer/coc/ programstandards2012.ashx. Accessed September 1, 2015. 6. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Distress Management. v2.2015. http:// www.nccn.org/professionals/physician_gls/pdf/distress.pdf. Accessed September 1, 2015. 7. National Comprehensive Cancer Network. NCCN practice guidelines for the management of psychosocial distress. Oncology (Williston Park). 1999;13(5A):113-147. 8.Canadian Association of Psychosocial Oncology (CAPO). National Standards for Psychosocial Oncology. Toronto, Ontario, Canada: CAPO; 1999. 9. Pirl WF, Muriel A, Hwang V, et al. Screening for psychological distress: a national survey of oncologists. J Supp Oncol. 2007;5(10):499-504. 10. Wagner LI, Spiegel D, Pearman T. Using the science of psychosocial care to implement the new American College of Surgeons commission on cancer distress screening standard. J Natl Compr Canc Netw. 2013; 11(2):214-221. 11. American College of Surgeons website. About CoC accreditation. https://www.facs.org/quality%20programs/cancer/accredited/about. Accessed September 3, 2015. 12. Grassi L, Watson M; IPOS Federation of Psycho-Oncology Societies’ co-authors. Psychosocial care in cancer: an overview of psychosocial programmes and national cancer plans of countries within the International Federation of Psycho-Oncology Societies. Psychooncology. 2012; 21(10):1027-1033. 13. Gouveia L, Lelorain S, Brédart A, et al. Oncologists’ perception of depressive symptoms in patients with advanced cancer: accuracy and relational correlates. BMC Psychol. 2015;3(1):6. 14. McDonald MV, Passik SD, Dugan W, et al. Nurses’ recognition of depression in their patients with cancer. Oncol Nurs Forum. 1999;26(3):593-599. 15. Passik SD, Dugan W, McDonald MV, et al. Oncologists’ recognition of depression in their patients with cancer. J Clin Oncol. 1998;16(4):1594-1600. 16. Howell D, Keller-Olaman S, Oliver T, et al; Canadian Partnership Against Cancer (Cancer Journey Action Group); Canadian Association of Psychosocial Oncology. A Pan-Canadian Practice Guideline: Screening, Assessment and Care of Psychosocial Distress (Depression, Anxiety) in Adults with Cancer. Toronto, ON, Canada: Canadian Association of Psychosocial Oncology; 2010. http://www.capo.ca/wp-content/uploads/2011/07/ENGLISH_Depression_Anxiety_Guidelines_for_Posting_150611.pdf. Accessed September 1, 2015. 17. Andersen BL, DeRubeis RJ, Berman BS, et al; American Society of Clinical Oncology. Screening, assessment, and care of anxiety and depressive symptoms in adults with cancer: an American Society of Clinical Oncology guideline adaptation. J Clin Oncol. 2014;32(15):1605-1619. 18. Kremer LC, Mulder RL, Oeffinger KC, et al; International Late Effects of Childhood Cancer Guideline Harmonization Group. A worldwide collaboration to harmonize guidelines for the long-term follow-up of childhood and young adult cancer survivors. Ped Blood Cancer. 2013;60(4):543-549. 19. Mulder RL, Kremer LC, Hudson MM, et al; International Late Effects of Childhood Cancer Guideline Harmonization Group. Recommendations for breast cancer surveillance for female survivors of childhood, adolescent, and young adult cancer given chest radiation. Lancet Oncol. 2013;14(13):e621-e629. October 2015, Vol. 22, No. 4 20. Donovan KA, Jacobsen PB. Progress in the implementation of NCCN guidelines for distress management by member institutions. J Natl Compr Canc Netw. 2013;11(2):223-226. 21. Jacobson JO, Neuss MN, McNiff KK, et al. Improvement in oncology practice performance through voluntary participation in the Quality Oncology Practice Initiative. J Clin Oncol. 2008;26(11):1893-1898. 22. Jacobsen PB, Shibata D, Siegel EM, et al. Evaluating the quality of psychosocial care in outpatient medical oncology settings using performance indicators. Psychooncology. 2011;20(11):1221-1227. 23. Neuss MN, Desch CE, McNiff KK, et al. A process for measuring the quality of cancer care: The Quality Oncology Practice Initiative. J Clin Oncol. 2005;25(25):6233-6239. 24. Jacobsen PB, Wagner LI. A new quality standard: the integration of psychosocial care into routine cancer care. J Clin Oncol. 2012;30(11):1154-1159. 25. Jacobsen PB, Kadlubek P. Change over time in quality of psychosocial care: results from the Quality Oncology Practice Initiative. Presented at: American Society of Clinical Oncology Annual Meeting; Chicago, IL: 2010. 26. Jacobsen PB, Lee JH, Fulp W, et al. Florida initiative for quality cancer care: changes in psychosocial quality of care indicators over a 3-year interval. J Oncol Pract. 2015;11(1):e103-e109. 27. Holland JC, Watson M, Dunn J. The IPOS new International Standard of Quality Cancer Care: integrating the psychosocial domain into routine care. Psychooncology. 2011;20(7):677-680. 28. Dudgeon D, King S, Howell D, et al. Cancer Care Ontario’s experience with implementation of routine physical and psychological symptom distress screening. Psychooncology. 2012;21(4):357-364. 29. Mitchell AJ, Lord K, Slattery J, et al. How feasible is implementation of distress screening by cancer clinicians in routine clinical care? Cancer. 2012;118(24)s:6260-6269. 30. Shimizu K, Ishbashi Y, Umezawa S, et al. Feasibility and usefulness of the ‘Distress Screening Program in Ambulatory Care’ in clinical oncology practice. Psychooncology. 2010;19(7):718-725. 31. Zebrack B, Kayser K, Sundstrom L, et al. Psychosocial distress screening implementation in cancer care: an analysis of adherence, responsiveness, and acceptability. J Clin Oncol. 2015;33(10):1165-1170. 32. Bauwens S, Baillon C, Distelmans W, et al. Systematic screening for distress in oncology practice using the Distress Barometer: the impact on referrals to psychosocial care. Psychooncology. 2014;23(7):804-811. 33. Forsythe LP, Rowland JH, Padgett L, et al. The cancer psychosocial care matrix: a community-derived evaluative tool for designing quality psychosocial cancer care delivery. Psychooncology. 2013;22(9):1953-1962. 34. National Cancer Institute Community Cancer Centers Program. NCCCP cancer palliative care matrix assessment tool. Updated March 2010. http://ncccp.cancer.gov/NCCCP-PCMatrix-bereavement.pdf. Accessed September 1, 2015. 35. Passalacqua R, Annunziata M, Borreani C, et al. Feasibility of a quality improvement strategy integrating psychosocial care into 28 medical cancer centers (HuCare project). Support Care Cancer. 2015. Epub ahead of print. 36. Pirl WF, Fann JR, Greer JA, et al. Recommendations for the implementation of distress screening programs in cancer centers: report from the American Psychosocial Oncology Society (APOS), Association of Oncology Social Work (AOSW), and Oncology Nursing Society (ONS) Joint Task Force. Cancer. 2014;120(19):2946-2954. 37. Blayney DW, McNiff KK, Eisenberg PD, et al. Development and future of the American Society of Clinical Oncology’s Quality Oncology Practice Initiative. J Clin Oncol. 2014;32(35):3907-3913. 38. Jacobsen PB, Shibata D, Siegel E, et al. Initial evaluation of quality indicators for psychosocial care of adults with cancer. Cancer Control. 2009;16(4):328-334. 39. Hendrick SS, Cobos E. Practical model for psychosocial care. J Oncol Pract. 2010;6(1):34-36. 40. Green LW, Kreuter MW. Health Promotion Planning: An Educational and Ecological Approach. 4th ed. New York: McGraw-Hill; 2005. 41. Schofield P, Carey M, Bonevski B, et al. Barriers to the provision of evidence-based psychosocial care in oncology. Psychooncology. 2006;15(10):863-872. October 2015, Vol. 22, No. 4 Cancer Control 449 Integrating palliative care has not always extended to rural areas; however, some research is focusing on future progressive solutions. Photo courtesy of Lisa Scholder. Masculine, 14" × 22". Systematic Review of Palliative Care in the Rural Setting Marie A. Bakitas, DNSc, CRNP, Ronit Elk, PhD, Meka Astin, MPH, Lyn Ceronsky, DNP, GNP, Kathleen N. Clifford, MSN, FNP-BC, J. Nicholas Dionne-Odom, PhD, RN, Linda L. Emanuel, PhD, MD, Regina M. Fink, RN, PhD, Elizabeth Kvale, MD, Sue Levkoff, ScD, MSW, Christine Ritchie, MD, MSPH, and Thomas Smith, MD Background: Many of the world’s population live in rural areas. However, access and dissemination of the advances taking place in the field of palliative care to patients living in rural areas have been limited. Methods: We searched 2 large databases of the medical literature and found 248 relevant articles; we also identified another 59 articles through networking and a hand search of reference lists. Of those 307 articles, 39 met the inclusion criteria and were grouped into the following subcategories: intervention (n = 4), needs assessment (n = 2), program planning (n = 3), program evaluation (n = 4), education (n = 7), financial (n = 8), and comprehensive/systematic literature reviews (n = 11). Results: We synthesized the current state of rural palliative care research and practice to identify important gaps for future research. Studies were conducted in the United States, Australia, Canada, Africa, Sweden, and India. Two randomized control trials were identified, both of which used telehealth approaches and had positive survival outcomes. One study demonstrated positive patient quality of life and depression outcomes. Conclusions: Research to guide rural palliative care practice is sparse. Approaches to telehealth, community–academic partnerships, and training rural health care professionals show promise, but more research is needed to determine best practices for providing palliative care to patients living in rural settings. Introduction In 1990, the World Health Organization revised its comprehensive Cancer Pain Relief and Palliative Care report to include palliative care.1 Since then, strides have been made worldwide in providing patients with cancer access to palliative care, and professional medi- From the School of Nursing (MAB, JND-O), Center for Palliative and Supportive Care (MAB, EK), and School of Public Health (MA), University of Alabama at Birmingham, Birmingham, Alabama, College of Nursing (RE) and College of Social Work (SL), University of South Carolina, Columbia, South Carolina, University of Minnesota Medical Center (LC), Minneapolis, Minnesota, St Luke’s Mountain States Tumor Institute (KNC), Boise, Idaho, Buehler Center on Aging, Health and Society (LLE), Northwestern’s Feinberg School of Medicine, Chicago, Illinois, University of Colorado Hospital and University of Colorado College of Nursing (RMF), Aurora, Colorado, University of California (CR), San Francisco, California, and Johns Hopkins Medical Institutions and Sidney Kimmel Comprehensive Cancer Center (TS), Baltimore, Maryland. Submitted July 6, 2015; accepted October 12, 2015. Address correspondence to Marie A. Bakitas, DNSc, CRNP, School of Nursing, Center for Palliative and Supportive Care, University of Alabama at Birmingham, 1701 University Boulevard, Birmingham, AL 35233. E-mail: mbakitas@uab.edu Dr Bakitas receives support from R01NR011871-01 and the American Cancer Society Research Scholar Grant. Dr Dionne-Odom is a postdoctoral fellow supported by the University of Alabama at Birmingham Cancer Prevention and Control Training Program (5R25CA047888) and by a National Palliative Care Research Center Junior Faculty Career Development Award. Dr Elk receives support from NIH/NIA: 1R21AG046772-01A1. No significant relationships exist between the other authors and the companies/organizations whose products or services may be referenced in this article. 450 Cancer Control October 2015, Vol. 22, No. 4 cal organizations have recommended early palliation for patients with advanced cancer.2-7 However, access and the dissemination of palliative care advances to the 40% to 60% of the global population who live in rural areas have been limited.8 Although persons with cancer are more likely than those with other, nononcological, progressive, and lifelimiting illnesses to receive palliative care, those with cancer living in rural communities continue to be underserved.9 The limited focus on palliative care in the rural setting is evident in the lack of guidance by national organizations to address the unique challenges and barriers faced by these patients and health care professionals.10 Clinical guidelines on quality palliative care from the National Consensus Project do not contain the term rural nor do they address how these standards should be applied in rural settings.11 Not only must performance indicators of palliative care need to be modified, but the reality is that many rural health care facilities do not have access to any type of specialty oncology or palliative care resources.10 Thus, the purpose of this paper is to synthesize empirical evidence, much of which has focused on persons with cancer, to describe the current practice and state of research relating to palliative care in the rural setting, and to identify important gaps for future research. Methods We conducted a systematic review of 2 large databases from January 1990 to February 2014. The search revealed 248 relevant articles and we identified an additional 59 articles through networking and a hand search of reference lists. We screened titles and abstracts of 307 articles and assessed the full text (focused on methodology sections to identify original research) of 225 articles. Thirty-nine articles met the inclusion criteria of being a research study or systematic review and had a rural focus. Papers meeting the inclusion criteria were included regardless of study quality so we could obtain the broadest possible representation of the state of research. Articles were grouped into 7 categories and 1 or 2 authors reviewed and synthesized each category. Studies reports represented the United States (n = 13), Australia (n = 7), Canada (n = 4), Africa (n = 2), Sweden (n = 1), and India (n = 1). Category definitions and summaries of the studies are described in Tables 1 and 2.12-49 Intervention Studies Four papers representing 2 randomized controlled trials (RCTs) were identified.12-15 Both RCTs offered early palliative care approaches to patients with advanced cancer and their family caregivers using a telehealth/ telephonic intervention. The settings, which were rural New Hampshire and Vermont outpatient cancer clinics that included a US Veterans Affairs Medical Center, October 2015, Vol. 22, No. 4 were the same for both studies.12,13 Each study generated separate papers focused on patient or caregiver outcomes.12-15 Positive patient quality of life (QOL), depression and survival and positive caregiver outcomes were reported.12,13,15 Needs Assessment Two US studies focused on needs assessments and the development of rural palliative care.16,17 Ceronsky et al17 focused on palliative care quality practices and utilized a learning collaborative methodology to assist communities to establish and strengthen palliative care capacity in rural Minnesota. A program-development evaluation survey assessed the benefits of the Minnesota Rural Palliative Care Initiative and satisfaction with participation.17 All the program components were rated well and an increase was seen in participant knowledge of pain management and goals of care discussions.17 Fink et al16 used an investigator-developed needs assessment instrument to determine awareness, knowledge, barriers, and access to resources of palliative care services in 236 Rocky Mountain–area rural hospitals. They found that 99% of health care professionals were familiar with the palliative care concept, 76% had a contract with a local hospice organization, 72% participated in a program to promote advancecare planning, 56% had access to palliative care resources, and 72% had palliative care education offered in the past year.16 Program Planning Three studies addressed program planning for rural palliative care services and programs; 2 were conducted in Australia and 1 in Canada.18-20 The Australian studies found that palliative care had moved from being marginalized to a key component of rural health care delivery.18,19 Blackford and Street18 developed and evaluated the effectiveness of a model to improve advance-care planning in the community setting. Their model consisted of 5 domains: servicelevel governance, advance-care planning education, advance-care planning documentation, community engagement, and quality processes.18 They found that an advance-care planning model is feasible in the rural community and should focus on advance-care planning conversations and involve families rather than just advance-care planning completion rates.18 Phillips et al19 evaluated the applicability of the Predisposing, Reinforcing, Enabling, Causes in, Educational Diagnosis and Evaluation (PRECEDE) and Policy, Regulatory, Organizational Constructs in Educational and Environmental Development (PROCEED) model in the development of targeted, nursing-led chronic illness interventions. They studied an aging population with rural, unmet palliative care needs and a disadvantaged urban community at high risk for cardiovascular disCancer Control 451 Table 1. — Selected Studies of Palliative Care in Rural Settings Study Design/ Intervention Sample Setting Measure Outcome Comment Intervention Bakitas12 Prospective RCT ENABLE phonebased coaching vs usual oncology care 322 NH/VT Outpatient cancer clinics and VAMC FACIT-Pal CES-D Edmonton Symptom Assessment Scale Resource Use Improved QOL and depression (both P = .02) Trend toward improved symptoms (P = .06) One of the first studies to describe benefits of early palliative care O’Hara14 Prospective RCT Patient-directed ENABLE phonebased coaching vs usual oncology care 222 NH/VT Outpatient cancer clinics and VAMC Caregiver burden MBCBS After-death interview Complicated grief No differences in caregiver burden Higher caregiver objective and stress burden related to lower patient QOL No specific intervention offered to these caregivers, but they were allowed to participate in patient intervention Bakitas13 Prospective, wait-control RCT ENABLE phonebased coaching at diagnosis vs 3-mo delayed 207 NH/VT Outpatient cancer clinics and VAMC CES-D FACIT-Pal QUAL-E Survival Resource use Immediate group had improved survival (15%, P = .38) compared with delayed group at 1y Failure to achieve recruitment target may have impacted ability to demonstrate differences between groups DionneOdom15 Prospective, wait-control RCT ENABLE phone-based coaching at diagnosis vs 3-mo delayed 122 NH/VT Outpatient cancer clinics and VAMC QOL-CG CES-D MBCBS Immediate group had improved depression at 3 mo (P = .02); decedent caregivers had improved depression and subjective burden (both P = .01) — Needs Assessment Ceronsky17 Quality improvement/learning collaborative to assist communities to establish/ strengthen palliative care in rural Minnesota using theory and NQF preferred practices 10 Rural Minnesota MRPCI Program development Evaluation survey to assess benefit of MRPCI and satisfaction with participation Teams grew from 1 to 6 All components of program rated well Increased knowledge of pain management by 73% and goals of care discussion by 81% 10 teams represented 64 organizations; used community capacity development theory Fink 16 Descriptive, determine awareness, knowledge, barriers, and resources of palliative care services in rural hospitals 374 236 Rocky Mountain rural hospitals (< 100 beds) in Montana, Wyoming, Utah, New Mexico, Colorado, Kansas, and Nebraska Investigator-developed qualitative/quantitative 7-section needs assessment instrument covering current palliative care services, professional education programs, desired learning methods, satisfaction with and barriers to palliative care, community resources, and populations needing assistance 99% familiar with palliative care concept 76% had a contract with hospice 72% participated in advancecare planning program 56% had access to palliative care resources 9% had board certified physicians in hospice and palliative medicine 72% offered palliative care education in past year Ethical issues and psychosocial support least satisfied Barriers: Patients avoiding discussions of dying and inadequate policies and education 36 surveys did not list location 40% response rate continued on next page 452 Cancer Control October 2015, Vol. 22, No. 4 Table 1. — Selected Studies of Palliative Care in Rural Settings (continued) Study Design/ Intervention Sample Setting Measure Outcome Comment Multisite, action research to design and evaluate the feasibility of an advance-care planning program 3 community sites n = 611 (urban) n = 460 (urban) n = 186 (regional) 3 community palliative care services: 1 regional and 2 metropolitan, urban in Victoria, Australia Client and service management audits (pre- and post-implementation of the program/model Key informant interviews (n = 9) Development and evaluation of efficacy of a model to improve advance care planning in the community setting Model to implement advance care planning in the community is feasible Documentation of advance care planning discussions with clients and families is a more useful outcome than completing advance-care planning Community palliative care services needs to engage with local communities Leadership essential ingredient to change the process Reflective case study approach to demonstrate the applicability of model to the development of nursing-led chronic illness interventions 2 case studies/ populations Regional coastal Australian aging population with rural, unmet palliative care needs Urban area in Sydney, Australia: Disadvantaged urban community at high risk for cardiovascular disease PRECEDE-PROCEED model used Needs assessment conducted (social, epidemiological, behavioral, environmental, educational, and ecological) Data shaped development of a multifaceted intervention focusing on increasing aged care personnel’s palliative care capacity, access to resources, development of evidence-based guidelines, and evaluation Assess feasibility of pilot study and explore symptom, cost, and satisfaction outcomes 44 initial consultations and 28 followup visits Canada Videoconferencing to provide specialist palliative care and radiotherapy consultation to those with cancer living in rural settings and explore symptoms, cost, and satisfaction outcomes Videoconferencing is feasible, may improve symptoms, results in cost savings, and satisfactory to patients and HCPs 1 case study Rural and remote Griffith, Australia Evaluation of Griffith Area Palliative Care Service model Key elements included: • 24-h access • Governance and staffing • Case management review • Enhanced primary care • Information management • Outcomes and evaluation • Weekly multidisciplinary team meeting Coordinated integrated application of existing resource Investment of new resources Formal evaluation Program Planning Blackford18 Phillips19 Watanabe20 ­ — — — Program Evaluation Hatton21 2-y pilot project delivering palliative care services Plans for future dissemination identified continued on next page October 2015, Vol. 22, No. 4 Cancer Control 453 Table 1. — Selected Studies of Palliative Care in Rural Settings (continued) Study Design/ Intervention Sample Setting Measure Outcome Comment Program Evaluation (continued) Wilkes22 Descriptive evaluation of a pilot project of a palliative care telephone call service 48 HCPs 21 afterhours telephone support nurses New South Wales, Australia Telephone logbook (12 calls), text analysis of reflective journals, questionnaire, interviews Major themes: • Program preparation and introduction • After-hours telephone support service for families and HCPs • Nurse experiences (personal impact and support) Lack of HCP knowledge about service Bensink 23 Evaluation of the acceptability of videotelephony/ webcam 17 Tertiary pediatric oncology service in Brisbane, Australia Interview (face to face or telephone) Cost analysis 92% participation rate Families receiving videotelephone calls found them to be more useful than a telephone call 2 RCTs previously attempted and abandoned following difficulty with family recruitment Evaluation of videotelephony was conducted Logie24 Program evaluation 8 5 hospices 3 homebased care organizations Zambia Multiple methods rapid field evaluation: • Desk surveys • Facility interviews • Data from 2 field visits (practice observation, trainee feedback, interviews with key personnel and funders) Program enhancement with modest funding included: • Training program (rural and urban) • Improved access to morphine and other drugs • Increased government lobbying to support palliative care and hospice and to improve standards For palliative care to thrive in a resource poor country, public health system integration is crucial and long-term funding needed Smith31 Qualitative program evaluation of providing specialists in 2 rural community hospitals 6,958 audited patient charts pre-RCOP and 7,572 post-RCOP RCOP of the Massey Cancer Center Medical College of Virginia No formal measurement; anecdotal observations include ability to keep patients with cancer in their local communities Rural practitioners able to safely administer cancer therapy Focused on outcomes for local clinicians Thulesius25 Comparison of learner-centered education program and control group 460 Rural areas in Sweden Intervention and control groups sepa-rated by 20-mile-wide rural district Matched in terms of demography and home care structure 20-item attitude questionnaire HADS administered to staff Postintervention: Significant differences on 17 of 20 items between 2 groups Preintervention: Significantly higher in education group Postintervention: Significantly lower in education group Improved attitude about care and improved mental health well-being of staff Education program was evidence-based, mixing small group work with lectures, seminars, and discussion Objective was to produce local guidelines for end-of-life care Only study reviewed with control group Education continued on next page 454 Cancer Control October 2015, Vol. 22, No. 4 Table 1. — Selected Studies of Palliative Care in Rural Settings (continued) Study Design/ Intervention Sample Setting Measure Outcome Comment Education (continued) Retrospective analysis of the Ontario Ministry of Health educational palliative care program delivered in last 8y 353 Pre- and postdesign 14 workshops for general practitioners tailored to specific patient symptoms 149 Kaufman28 Prospective design study of educational presentations to HCPs (nurses = half-day conference; physicians = grand rounds) Easom29 Pre- and postdesign study of educational presentations to rural nurses and licensed practical nurses in assisted-living and nursing home environments Kelley 26 Reymond27 Ontario Questionnaires (26 questions) 83% said training very significant compared with other sources of learning 87% reported practice of palliative care changed 91% shared palliative care knowledge with others 68% reported palliative care delivery in their community much better than prior to training 70% now had palliative care team (compared with 9% prior to training) Remote areas in Australia Cost of training Evaluation of education and clinical objectives Goal to evaluate intervention aimed at increasing knowledge and capacity of palliative care 95% general practitioner satisfaction with workshop in teaching palliative care skills (91% for nurses and other HCPs) Confidence in managing palliative care cases increased from 2.9 to 3.9 (5-point scale) HCPs reported improvement in confidence at 3 mo 27 New Mexico Change in hospice utilization pattern: • Subsequent referral to hospice • Patient referral pattern • Length of stay • Diagnosis at admission • Nursing home referral pattern Significant increase in hospice utilization (33 to 61) 113/254 eligible for hospice were referred (65%) Increase in referrals by community nursing homes (but not other sources) • Not significant in time in days in hospice • Not significant in terms of diagnosis • Significant increase in frequency of referral from 2 nursing homes 9 Assisted living facility in southeastern United States End-of-life attitudes survey End-of-life knowledge assessment Open-ended questions Post-test results significantly higher than pre-test knowledge on end-of-life care Most knowledge growth on response to pain medication, administration of opioid analgesics for pain, and interventions to relieve nausea Significant difference in attitudes — — Goal to increase hospice utilization in rural community Effective for changes in referral by nursing homes alone (no change in physician referrals) Training conducted by local HCPs at very low cost — continued on next page October 2015, Vol. 22, No. 4 Cancer Control 455 Table 1. — Selected Studies of Palliative Care in Rural Settings (continued) Study Design/ Intervention Sample Setting Measure Outcome Comment Education (continued) Determine staff needs for palliative care education, then develop 15-h interprofessional curriculum Program piloted in 3 facilities 128 Ontario (remote, rural, and longterm care facilities) Identify 3 most impor- Highest ranking topics: stress tant learning needs and management for staff (73%), preferred format for individual and family (71%), understanding emotional needs of dying person (67%) Majority preferred education in face-to-face format, liked interdisciplinary focus, teambuilding, felt empowered by program Approach can serve as a model for palliative care education in other rural areas Smith31 Pre- and postfinancial analysis of RCOP 1 site 3 rural clinics serving 3–5 counties in Virginia Chart audits after 3 y of operation (postRCOP) compared with 2-y results (pre-RCOP) Profitable for rural hospitals Revenue-neutral for academic centers Cost saving for society Successful enough to be a required part of all comprehensive cancer center programs Desch32 Pre- and postfinancial data analysis of RCOP 1 site Low-income areas in rural Virginia Main outcome measures: • Costs (estimated reimbursement from all sources) • Revenues • Contribution margins • Profit (or loss) Rural hospitals generated > $500,000 USD/y after RCOP Total cost per patient in network decreased from $10,233 USD to $4,392 USD (57% decrease) Programmatic part of all National Cancer Institute centers Uys 33 3-mo, multisite home-based care project providing palliative care for patients with AIDS 7 sites rural = 2 periurban = 2 urban = 3 Underserved in South Africa Evaluation of costs: • Setup (training, equipment, and planning) • Cost per site • Site operating cost (total and average per patient) • Average hospital inpatient • Hospital outpatient • Primary care clinic costs per participating patient Palliative home-based care increased in rural areas where a vehicle is required for staff transport Impossible to know whether home-based care adds services or can cost-effectively substitute for hospital services Cassel34 Analysis of Rappahannock Rural Palliative Care Program 1 site Northern Virginia, located in a 5-county rural farming area Seacoast area on Chesapeake Bay Collected data from consults, physician billing, and receipts to calculate hospital charges for patients treated with concurrent palliative care Cost per day decreased to $400 USD/y (25% of total) Generated $80,000–100,000 USD in savings in reduced hospital charges and cost per year Single site pilot survey of patientcaregiver dyads 11 Trivandrum, Kerala, India Southwest coast of India Pilot study of economic impact and openness to training 100% reported decreased family earnings Families surveyed averaged $1,082 USD of debt 8 of 11 caregivers open to training KortesMiller 30 Financial Emanuel38 — — continued on next page 456 Cancer Control October 2015, Vol. 22, No. 4 Table 1. — Selected Studies of Palliative Care in Rural Settings (continued) Study Design/ Intervention Sample Setting Measure Outcome Comment Project has expanded Did not take into account cost avoidance or hospitalization cost avoided Medicare hospice benefits: cost equivalent to US Medicare hospice benefits and lower than average alternate level of care and hospital costs within Ontario, Canada Education (continued) Klinger35 Analyze resource utilization and costs of Niagara West End-of-Life Care Project over a 15-mo period 3 small towns Public health system of Ontario, Canada Economic evaluation to establish evaluations in monetary units and Expenditure Panel Survey Total costs for all patient-related were $1,625,658.07 CAN ($17,112.19 CAN/patient and $117.95 CAN/patient/d) Costs within parameters of Medicare hospice benefits Simon37 Pilot survey of working-age adults with a family member with a serious/long-term illness 81 workingaged US adults Web-based US adults Cost of illness-related health care Changes in economic behaviors Changes in employment or education behaviors Mean reported cost of medical bills paid by respondents ($17,108 USD) 29.4% of respondents sold possessions as a result of family illness 42.4% reduced work hours as a result of family illness Bradford36 Cost minimization 95 home analysis comparing video conactual costs of sultations HTP consultations with estimated potential costs associated with face-to-face consultations occurring by either hospitalbased consultations in outpatient department or home visits from pediatric palliative care specialists Royal Children’s Hospital (Brisbane, Australia) Data from 95 home video consultations occurring over 2 y Included costs associated with projected: clinician time and travel, costs reimbursed to families for travel through the Patients Travel Subsidy scheme, hospital outpatient clinic costs, project coordination, and equipment and infrastructure costs Mean costs per consultation calculated for each approach Air travel (n = 24) significantly affected results Mean cost of HTP intervention was $294 USD and required no travel Estimated mean cost per consultation in the hospital outpatient department was $748 USD Mean cost of home visits per consultation was $1,214 USD Face-to-face consultations are gold standard of care, but families located at a distance from the hospital may find video consultation in the home to be an effective and cost efficient method to attend a consultation Video consultation in the home ensures equity of access to services and minimum disruption to hospital-based palliative care teams P ≤ .05 is considered statistically significant. CAN = Canadian dollar, CES-D = Center for Epidemiological Studies Depression Scale, ENABLE = Educate, Nurture, Advise Before Life Ends, FACIT-Pal = Functional Assessment of Chronic Illness Therapy-Palliative, HADS = Hospital Anxiety and Depression Scale, HCP = health care professional, HTP = Home Telehealth Program, ICHC = integrated community home-based care, MBCBS = Montgomery Borgatta Caregiver Burden Scale, MRPIC = Minnesota Rural Palliative Care Initiative, NH/VT = New Hampshire/Vermont, NQF = National Quality Forum, PRECEDE = Predisposing, Reinforcing, Enabling Constructs in Education, Diagnosis, and Evaluation, PROCEED = Policy, Regulatory, Organizational Constructs in Educational and Environmental Development, QOL = quality of life, QOL-CG = Quality of Life Caregiver Scale, QUAL-E = Quality of Life at the End of Life Scale, RCOP = Rural Cancer Outreach Program, RCT = randomized controlled trial, USD = US dollar, VAMC = US Veterans Affairs Medical Center. ease.19 They suggested the PRECEDE-PROCEED model could be used to guide nursing-led interventions in existing health care environments.19 Watanabe et al20 evaluated the feasibility of videoconferencing to provide specialist palliative care, radiotherapy consultations, and to explore symptoms in rural Canadian persons with cancer. They found videoconferencing to be feasible, suggested that its use may improve symptoms and result in cost savings, as well as inOctober 2015, Vol. 22, No. 4 crease satisfaction for patients, families, and health care professionals.20 Program Evaluation Three Australian studies and 1 Zambian study evaluated various, rural, palliative care delivery approaches.21-24 Hatton et al21 evaluated a pilot collaborative project among multiple agencies in Australia to meet the challenges of providing palliative care services in rural and Cancer Control 457 Table 2. — Selected Systematic Reviews of Rural Palliative Care in Rural Settings Study Purpose Findings Evans Conduct systematic literature review of studies examining organization of rural palliative care and views of professionals in rural areas No case-control, cohort, randomized controlled trials, or meta-analyses were found Majority of studies based out of Australia (n = 16) Role of primary care discussed in 12 studies Problems reported in the provision of symptom control for patients Issues with education, training, and emotional support for professionals providing palliative care in rural settings Accessing specialist services was reportedly difficult for professionals Professionals and rural families described difficulties in accessing information Developments in information technology were mentioned in a few studies as possible solutions Need perspectives from rural primary care professionals about the optimal organization of palliative care in the rural setting Hughes 40 Identify needs of rural-dwelling patients with cancer and their caregivers in delivery of palliative care All studies from developed countries No studies specifically about ethnic minorities Noted difficulty of comparing studies due to differing methodologies and contexts Rural caregivers may have additional care demands placed on them Information needs may be higher in rural vs urban patients Geographical distance a major variable in care Jennett41 Review of readiness models for rural telehealth Four distinct models identified for readiness assessment Each model discussed various themes essential for telehealth readiness Three themes common to each discussions: appreciation of practice context, strong leadership, perceived need to improve practice Combining e-health and telehealth with health informatics Theories of change, diffusion of innovations, components of telehealth readiness tools (eg, patient, public, health care professional, organization, system) could be reviewed and refined for application to e-health Wilson42 Assess challenges and other important issues/ circumstances involving planning and providing EOL care in rural areas Most research was single site, small sample, and exploratory Identified and described differences between EOL care in urban and rural settings, assessed EOL needs and wishes of terminally ill or dying persons, their family members, and health care professionals in rural areas, explored EOL education for rural EOL specialists EOL care as an essential service in rural communities Integration of EOL care into rural health care settings Family caregivers must be provided with more information and support (eg, home-based nursing care) Improved linkages to specialized palliative care necessary for continuing education Prepare and support rural health care professionals while delivering EOL care Steers 43 Determine role played by UK community hospitals in provision of palliative care Many UK community hospitals have resources to counter inequalities in access to general palliative care More prospective research using qualitative methods involving patients, caregivers and nurses required to understand complexities of providing palliative care Cox44 Literature review of quality of care in rural areas Rural communities more different than alike Rural issues differ from those in urban settings Research in rural health facilities to determine best practices for patient safety and outcomes Must determine best practices for mental health and effective smoking cessation programs and technological interventions Identification of mental health programs and services providing cost-effective quality care Robinson45 Identify, evaluate, and synthesize literature on rural palliative care Grouped into patient and caregiver perspectives, professional attitudes, knowledge, and practice issues, and health care services Body of research is small and eclectic Little evidence to inform palliative policy and service development in rural settings Coordinated programs required to develop adequate body of knowledge to support effective service and policy development 39 continued on next page 458 Cancer Control October 2015, Vol. 22, No. 4 Table 2. — Selected Systematic Reviews of Rural Palliative Care in Rural Settings (continued) Study Downing Purpose Findings Explore global developments in palliative care provision in rural settings since 2010, highlighting models, including challenges faced in establishing services Provision of care based on premise that individuals wish to die at home Communication is a key concept to palliative care Community volunteers and networks intrinsic to palliative care models of rural Sub-Saharan Africa Challenges to recruitment and sustainability of main clinical team Lack of rural generalist providers having access to specialist support Major challenge is the cost and time of travel to access care Care services need to be individually tailored to specific rural communities Use of community volunteers in care delivery may be effective Practical issues related to access and traversing geographical distances remain challenging Phillips 47 Review published studies evaluating impact of continuing professional development programs on ability of rural nurses to provide palliative care; inform the development of targeted learning activities for this population Evaluated programs involving rural nurses and focused on increasing care capabilities Evidence limited by the absence of randomized controlled trials Valuable insights into barriers and facilitators to engaging rural nurses in learning opportunities Continuing education to positively impact patient and family outcomes Optimize opportunities for web-based technologies by developing and maintaining computer competencies Investigation needed of impact of specialist clinical placements capabilities to provide palliative care among nurses Jang48 Examine opioid availability, caregiver burden, and use of health care resources at patient EOL by setting to determine optimal setting for palliation in Africa Recognizing and treating symptoms occurring at the EOL Research in Africa sparse; most from South Africa and Uganda Use of health care resources for palliative care at EOL More research on nurse prescribing training program, which is part of Uganda’s public morphine program O’Brien49 Discuss palliative care and EOL models of care for Aboriginal people in New South Wales, Australia Resistance to the idea of inpatient care due to Aboriginal Australians from their homeland and community Ceremonies that assist the spirit to leave the physical body and return to its sacred place common among Aboriginal people Lack of understanding of Aboriginal belief systems and their links to the land lead to a disconnect for health care professionals Aboriginal people must have culturally appropriate and locally accessible palliative care services because they have a sense of isolation and are disconnected culturally from accessing mainstream services Clarification of the social, emotional, spiritual, and cultural factors that influence decision making about accessing palliative care among Aboriginals 46 EOL = end of life. remote areas of Australia. Key elements of the model included enhanced primary care, 24-hour palliative care access, weekly multidisciplinary team meetings, case management review, and a formal outcomes evaluation of existing resources.21 Barriers comprised inequity in community palliative care support and access to ambulatory and home services for patients with cancer compared with those without cancer, but with no formal system for after-hours home nursing support, and lack of dedicated palliative care beds.21 Two studies evaluated technological support for palliative care in rural areas.22,23 Wilkes et al22 evaluated an after-hours, nurse-driven telephone support service and found that its availability reduced isolation for families caring for rural palliative care patients at home. Physicians and nurses were satisfied with its accessibility because they believed it decreased rehospitalization rates for seriously ill patients.22 October 2015, Vol. 22, No. 4 Bensink et al23 researched the acceptability of video telephone services to pediatric patients in regional and remote areas. This study team attempted 2 RCTs with this patient population; however, due to ethical constraints (eg, family reluctance to participate, patients too sick to participate) and technological issues (eg, blurred video, internet connection problems) the RCTs were not fully carried out.23 They suggested future research might include integration of a videotelephony model at the time of diagnosis with a life-threatening illness, rather than at the time of palliative care.23 One study evaluated a synergistic, multipronged palliative care initiative in Zambia, a country with a high HIV prevalence rate and poor access to care.24 Eight hospices and palliative care organizations in poor and rural areas were partially funded for 2 years, and an extensive training program of staff was iniCancer Control 459 tiated.24 A mixed-method analysis of outcome was conducted and determined that the palliative care environment in Zambia was strengthened with this approach.24 Funding enabled agencies to expand their services, more reliably offer morphine, provide gas for transportation, and support patient caregivers.24 Training enhanced staff confidence in caring for very ill and dying patients and their families, and raised competence and confidence rates were sustained 2 years later.24 However, once funding ended, services had to be curtailed, which led to the determination that the need for sustainable, long-term funding is essential to maintain success.24 Education Six studies evaluated palliative care services and education in rural patient settings.25-30 One study used a retrospective design and a control group.25 The studies used various education methods (didactic or experiential) and time allocation (half-day to multiple days).25-30 One study developed training methods by assessing the needs of staff and another tailored the training to specific patient symptoms (eg, pain, constipation, dyspnea, delirium).27,30 Training resulted in enhanced knowledge, and changes to the palliative program, including initiation of a palliative care team, increased hospice utilization, improved staff attitudes, and improved confidence in providing care.25-28 Most educational programs were low cost but yielded significant changes in study outcomes. Financial Eight studies analyzed financial data from rural-area palliative care programs: 4 were conducted in the United States, and 1 each in Canada, South Africa, Australia, and India.31-38 Smith et al31 conducted preand post-financial analyses of the Rural Cancer Outreach Program (RCOP) in 3 clinics that served 3 to 5 counties in Virginia. Chart audits were performed after 3 years of operation (post-RCOP) and results were compared with the 2 years preceding the study (pre-RCOP).31 Outcome measures included costs (estimated reimbursement from all sources), revenues, contribution margins, and profit (or loss) of the program.31 RCOP generated at least $1 million in profit for the rural hospital, even while expanding cancer and palliative services, and reduced the net cost per patient by about 40% due to better coordination.31 RCOP was deemed profitable for the rural hospitals, revenue-neutral for the academic centers (a marked increase in referrals, including 9% in nononcological cases, from the rural areas was offset by the poor payer mix, which included uninsured and underinsured patients), and cost saving for society; it has also continued for 25 years.31 Cassel et al34 performed an analysis of the Rap460 Cancer Control pahannock Rural Palliative Care Program located in a 5-county rural farming and seacoast area in northern Virginia. This study collected data from consultations, physician billing, and receipts, and the researchers calculated hospital charges for patients treated with concurrent palliative care.34 Klinger et al35 analyzed resource utilization and costs of the Niagara West End-of-Life Care Project during a 15-month period in rural Ontario, Canada. The project was a success because hospital costs decreased by more than $400 per day when palliative care was involved, the in-hospital death rate decreased, and hospice discharges increased.35 The net effect was a sustainable program with $80,000 to $130,000 savings per year (although this number does not include avoided rehospitalizations).35 Uys et al33 conducted a 3-month, multisite, homebased care project providing palliative care for South African patients with AIDS living in underserved areas. Evaluation of costs included setup (training, equipment and planning), cost per home-based care site, home-based care operating cost (total and average per patient), and average hospital inpatient, hospital outpatient, and primary care clinic costs per participating patient.33 It was not clear whether home-based care added services or was a cost-effective substitute for hospital services.33 Bradford et al36 conducted a cost-minimization analysis to determine the cost of a home telehealth consultation for pediatric palliative care patients compared with costs of either a face-to-face consultation at a hospital or a home visit from a palliative care service. The cost for the home telehealth consultation was cheaper than the other 2 options because the telehealth option required no travel (which substantially increased costs).36 For families living in rural areas who must sometimes travel long distances to get to a hospital, video consultation can help ensure equity in access to quality palliative care.36 Two studies from the same team — one focusing on India and the other in the United States — focused on the economic impact of a terminal illness and the feasibility of training caregivers as a means of stemming illness-related poverty.37,38 The pilot study conducted in India found that patients were forced to give up work as a result of their illness and, in the majority of families, caregivers had to change their work habits and many had to sell assets.38 Most families indicated that a trained caregiver would have reduced or prevented some of the household’s illness-related change, and most caregivers said they would be interested in becoming a trained caregiver.38 The second paper proposed a strategy that would simultaneously mitigate household financial pressure as a result of illness as well as train caregivers to provide care that would in turn address the workforce talent shortage.37 October 2015, Vol. 22, No. 4 Systematic Reviews Eleven papers were systematic reviews (see Table 2).39-49 Six of the reviews directly assessed palliative care in rural settings, and 5 assessed some aspect of rural palliative care, though this was not specifically part of the study purposes.39-49 Robinson et al50 identified and evaluated 79 studies to ascertain the strength of evidence available to inform public policy and guidelines and identify direction for future research. Identified studies were grouped into 3 categories: patient and caregiver perspectives (n = 24), professional attitudes, knowledge, and practice issues (n = 28), and health care services (n = 27).50 Our review of these studies concluded that little is known about experiences of rural patients and their caregivers because only 6 of the 24 studies directly assessed these perspectives. In addition, a need exists to better operationalize how palliative care specialists should integrate with rural primary care physicians. We also concluded that the medical literature is eclectic and lacks focused and sustained programs of research that specifically focus on developing and testing models of palliative care delivery in the rural setting.50 Discussion When death is imminent, no second chance exists to improve the quality of care for the patient and family.51 Nationally, a significant increase has been seen in palliative care programs attempting to “get it right the first time”; however, rural areas still have limited palliative services, thus resulting in an important disparity for seriously ill patients with cancer and those without cancer.9 Nevertheless, as is evident from this review, through community support, academic support and partnerships, telehealth, community advisers, and other creative strategies, providing expert care to seriously ill patients may be feasible, even in the most remote locations. Such success occurs not by bringing the patient to the urban experts, but by bringing palliative care expertise to the patient and/or ensuring that palliative care support becomes imbedded into the fabric of the rural community.17,18,21,24 Tailoring studies and methods to the unique aspects of a rural community likely resulted in most studies using a quality-improvement design because the primary goal of the initiative was to improve care in a particular area or system rather than for the purpose of creating generalizable knowledge. We acknowledge that publication bias is a limitation in our review, and we are aware of many unpublished examples of the successful integration of palliative care into rural communities. For example, 10 years ago, the Center to Advance Palliative Care, the National Hospice and Palliative Care Organization, and the National Rural Health Association partnered to compile Providing Hospice and Palliative Care in Rural and Frontier Areas, a comprehensive October 2015, Vol. 22, No. 4 toolkit with exemplars of rural palliative care programs.52 Key informants from 31 programs were interviewed, representing 5 rural regions across the United States.52 Yet, our literature search yielded few added information about their programs since that monograph was published.52 Nonetheless, the body of published work that we reviewed has yielded a number of important pearls and lessons. The first of the 2 RCTs, which used a telehealth/ telephonic approach in patients newly diagnosed with advanced cancer, was also the first RCT to demonstrate the benefits of palliative care in any locale compared with usual cancer care.12 Despite the positive rates of patient QOL, mood, and survival outcomes in the first RCT and positive care giver outcomes in the subsequent RCT, the results are difficult to generalize due to the racial/ethnic homogeneity of the study participants.15 Quality-improvement efforts have also demonstrated positive results in rural settings. In the Minnesota Rural Palliative Care Initiative, a needs assessment was combined with an active quality improvement project.17 These rural communities identified diverse community resources and a strong commitment to developing palliative care programs.17 Although palliative specialists were commonly not available, hospice programs were identified as a primary resource for enhancing clinical knowledge.17 Improving processes of care was an identified need and teams worked across settings to meet quality guidelines.17 One finding in this review is that the community is pivotal to any future plans for developing primary palliative care services in the rural area. This includes enhancing education within the community so that primary care clinicians may have the knowledge to integrate 24-hour palliative care access in the rural area to all patients with life-threatening illness, not only patients with cancer. Educational offerings for rural practitioners may be web-based or onsite intensives. Partnering with local hospices to assist with palliative care education for clinicians may be helpful because the ability of rural health care professionals to travel to conferences can be a barrier due to lack of time and insufficient workload relief.9 Cancer Care Manitoba maintains a website (www.cancercare.mb.ca) describing its long-standing Community Cancer Program Network. According to its website, the Community Cancer Program Network, in partnership with regional health authorities, has helped rural patients outside of Winnipeg, Canada, receive oncology care close to home in 1 of 15 community cancer programs. A primary palliative care model geared toward the rural setting might include the provision of palliative care through an existing home nursing–care agency, interdisciplinary team involvement as needed, difficult case review with access to tertiary palliative care serCancer Control 461 vices through videoconferencing, a formal assessment of existing resources, and subsequent outcomes evaluation. Of interest, in the rural Virginia Cancer/Palliative Outreach Program, educating rural health care professionals was a compelling reason for the retention of health staff.53 In addition, strategic alliances between rural health care professionals and academic centers made access to up-to-date consultations, telehealth, and experts easier.32 Limited studies, many of which have focused on rural cancer programs, have documented the cost savings/avoidance of employing palliative care services in rural areas.54,55 This body of work suggests that the impact of palliative care on health care costs and services is similar for both rural and urban populations.54,55 For example, the $400/day savings observed in the rural Virginia study is very similar to the $279 to $374/ day savings observed in contemporary, urban hospital studies.54 Furthermore, the increased use of home hospice always follows palliative care consultations55; the only concern may be that reduced occupancy may stress rural hospitals, especially if they cannot maintain a census of adequately insured patients. Palliative care may also positively impact household economics in rural and urban settings by keeping the patient and family caregivers economically productive, requiring less need for travel and time off from work.54,55 The 11 systematic reviews addressed some component of rural palliative care (either alone or combined with urban studies), but the purposes, quality, and methodological rigor considerably varied among them. Thus, any direct comparison, compilation, and formal ratings of evidence strength among the reviews were problematic. Nevertheless, a few common themes were noted: • Small and insufficient literature base to guide palliative care policy development in rural areas • Geographical distance and lack of access to palliative care specialists limit the integration of quality palliative and end-of-life care in rural health care settings • Stakeholder perspectives and “on-the-ground” involvement are essential to incorporating palliative care services to rural settings that are more dissimilar than homogeneous • Policy and delivery models of rural palliative care have yet to be fully developed and tested • Although promising, emerging telehealth and ehealth methods need further testing, with emphases on improving health technology, connectivity, and competencies of rural health care professionals Notable gaps in these reviews included assessments of symptom management practices in rural settings and palliative care for specific illness subtypes. Use of telehealth strategies was prominent as a way to bring palliative support to patients. This tech462 Cancer Control nology is ushering in a new era of medicine in which health care will move toward becoming a commodity among others that people can access from their home or mobile devices.56 It is powerful and will entail rethinking the core of medicine, namely the doctor–patient relationship. Its decentralized and electronically mediated nature and the likelihood of scaling up due to its convenience (thus driving greater use) is likely to make quality control and transparent accountability both more challenging and easier.57 The types of medical errors that can occur while using mobile health technologies will morph, and methods for reducing medical errors must be revised to keep up. The roles of family caregivers and local unskilled, semi-skilled, and professional providers and distance providers will all change, entailing adjustments to credentialing and maintenance of professional certification.58 The economics of care delivery and the form of reimbursement will likely change, requiring revised models for billing and insurance. Emerging new issues that can scarcely be predicted will require assessment of concomitant regulatory issues.59 Policymakers will need to keep a close eye on telehealth to keep pace with its rapid development. Given the ability of integrated care-delivery networks to increase access to high-quality, personcentered care, the time is now to develop and evaluate comprehensive models of telemedicine that include standardized telephone support, use of peripherals to assess vital signs and oxygenation status, and video care to assess and interact with patients who are in a place of crisis.56,60 These models warrant rigorous testing and attention to the unique challenges and needs faced by patients in rural areas.56,60 Other successful initiatives to expand palliative care expertise in low-resource and rural areas have included community partnerships and training local community clinicians, community health advisers, community health workers, and communication, behavioral health, and palliative care specialists.10,61 These initiatives can be time intensive due to the time and need for developing relationships with the cultural leaders and within the norms of the community.10,61 However, attempting to skip this critical step could result in efforts not relevant to or not embraced by community members. Regardless of the strategies employed, expanding palliative care into rural areas will take explicit planning and multicomponent, unique strategies. Conclusions Research has informed the development of professional guidelines and integration of the principles of palliation into oncology care, from the time of diagnosis to end of life among patients with curable cancers, highsymptom burden, and metastatic disease alike. However, the rural setting has created a barrier for these October 2015, Vol. 22, No. 4 advances to reach patients not located near specialty centers. This global issue has been recognized and efforts are being initiated to discover novel strategies to ensure that high-quality palliative and end-of-life care can reach those living in rural communities. Telehealth, community health workers, specialists trained in various health care fields, and other volunteers trained in the approaches of palliative care have shown promise in bringing complex comfort strategies to many remote regions of the world. In addition, the ability to educate all of the health care professionals working in rural settings presents challenges that are surmountable. Organized efforts do exist for bringing palliative care skills to rural health clinicians and care settings. Shortages of palliative care specialists are a reality, even in urban areas. Hence, every clinician should have a basic set of primary palliative care skills of communication, advance care planning, and symptom control to ensure high-quality care for all persons with cancer and their families. Acknowledgments: The authors wish to thank Karen Herman for coordinating the search and Kristen Allen and Claire Bourgeois for assistance with manuscript preparation. 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Smith TJ, Desch CE, Grasso MA, et al. The Rural Cancer Outreach Program: clinical and financial analysis of palliative and curative care for an underserved population. Cancer Treat Rev. 1996;22(suppl A):97-101. 32. Desch CE, Grasso MA, McCue MJ, et al. A rural cancer outreach program lowers patient care costs and benefits both the rural hospitals and sponsoring academic medical center. J Rural Health. 1999;15(2):157-167. 33. Uys L, Hensher M. The cost of home-based terminal care for people with AIDS in South Africa. S Afr Med J. 2002;92(8):624-628. 34. Cassel JB, Webb-Wright J, Holmes J, et al. Clinical and financial impact of a palliative care program at a small rural hospital. J Palliat Med. 2010;13(11):1339-1343. 35. Klinger CA, Howell D, Marshall D, et al. Resource utilization and cost analyses of home-based palliative care service provision: the Niagara West End-of-Life Shared-Care Project. Palliat Med. 2013;27(2):115-122. 36. Bradford NK, Armfield NR, Young J, et al. Paediatric palliative care by video consultation at home: a cost minimisation analysis. BMC Health Serv Res. 2014;14:328. 37. Simon MA, Gunia B, Martin EJ, et al. Path toward economic resilience for family caregivers: mitigating household deprivation and the health care talent shortage at the same time. Gerontologist. 2013;53(5):861-873. 38. Emanuel N, Simon MA, Burt M, et al. Economic impact of terminal illness and the willingness to change it. J Palliat Med. 2010;13(8):941-944. 39. Evans R, Stone D, Elwyn G. Organizing palliative care for rural populations: a systematic review of the evidence. Fam Pract. 2003;20(3):304-310. 40. Hughes PM, Ingleton MC, Noble B, et al. Providing cancer and palliative care in rural areas: a review of patient and carer needs. J Palliat Care. 2004;20(1):44-49. 41. Jennett PA, Gagnon MP, Brandstadt HK. Preparing for success: readiness models for rural telehealth. J Postgrad Med. 2005;51(4):279-285. 42. Wilson DM, Justice C, Sheps S, et al. Planning and providing endof-life care in rural areas. J Rural Health. 2006;22(2):174-181. 43. Steers J, Brereton L, Ingleton C. Palliative care for all? A review of the evidence in community hospitals. Int J Palliat Nurs. 2007;13(8):392-399. 44. Cox K, Mahone I, Merwin E. Improving the quality of rural nursing care. Annu Rev Nurs Res. 2008;26:175-194. Cancer Control 463 45. Robinson CA, Pesut B, Bottorff JL, et al. Rural palliative care: a comprehensive review. J Palliat Med. 2009. [Epub ahead of print] 46. Downing J, Jack BA. End-of-life care in rural areas: what is different? Curr Opin Support Palliat Care. 2012;6(3):391-397. 47. Phillips JL, Piza M, Ingham J. Continuing professional development programmes for rural nurses involved in palliative care delivery: an integrative review. Nurse Educ Today. 2012;32(4):385-392. 48. Jang J, Lazenby M. Current state of palliative and end-of-life care in home versus inpatient facilities and urban versus rural settings in Africa. Palliat Support Care. 2013;11(5):425-442. 49. O’Brien AP, Bloomer MJ, McGrath P, et al. Considering Aboriginal palliative care models: the challenges for mainstream services. Rural Remote Health. 2013;13(2):2339. 50. Robinson CA, Pesut B, Bottorff JL. Issues in rural palliative care: views from the countryside. J Rural Health. 2010;26(1):78-84. 51. Higginson IJ, Hearn J, Webb D. Audit in palliative care: does practice change? Eur J Cancer Care (Engl). 1996;5(4):233-236. 52. National Rural Health Association. Providing Hospice and Palliative Care in Rural and Frontier Areas. Leawood, KS: National Rural Health Association; 2005. 53. Smith TJ, Desch CE, Simonson CJ, et al. Teaching specialty cancer medicine in rural hospitals: the cancer outreach program as a model. J Cancer Educ. 1991;6(4):235-240. 54. Morrison R, Penrod JD, Cassel J, et al. Cost savings associated with us hospital palliative care consultation programs. Arch Intern Med. 2008;168(16):1783-1790. 55. Hughes MT, Smith TJ. The growth of palliative care in the United States. Annu Rev Public Health. 2014;35:459-475. 56. Adibi S, ed. Mobile Health: A Technology Road Map. Vol. 5. New York: Springer; 2015. 57. Emanuel LL; Working Group on Accountability. A professional response to demands for accountability: practical recommendations regarding ethical aspects of patient care. Ann Intern Med. 1996;124(2):240-249. 58. American Telemedicine Association website. http://www.american telemed.org. Accessed November 5, 2015. 59. American Hospital Association. Realizing the promise of telehealth: understanding the legal and regulatory challenges. TrendWatch. Published May 2015. http://www.aha.org/research/reports/tw/15may-tw-telehealth .pdf. Accessed November 5, 2015. 60. Kelley AS, Meier DE, eds. Meeting the Needs of Older Adults With Serious Illness. New York: Humana Press; 2014. 61. Kaasalainen S1, Brazil K, Kelley ML. Building capacity in palliative care for personal support workers in long-term care through experiential learning. Int J Older People Nurs. 2014;9(2):151-158. 464 Cancer Control October 2015, Vol. 22, No. 4 Palliative care benefits patients with cancer in the outpatient setting, and such care should be concurrently and routinely provided with oncology treatment. Photo courtesy of Lisa Scholder. Family. 16" × 24". New Frontiers in Outpatient Palliative Care for Patients With Cancer Michael W. Rabow, MD, Constance Dahlin, ANP-BC, ACHPN, Brook Calton, MD, Kara Bischoff, MD, and Christine Ritchie, MD, MSPH Background: Although much evidence has accumulated demonstrating its benefit, relatively little is known about outpatient palliative care in patients with cancer. Methods: This paper reviews the literature and perspectives from content experts to describe the current state of outpatient palliative care in the oncology setting and current areas of innovation and promise in the field. Results: Evidence, including from controlled trials, documents the benefits of outpatient palliative care in the oncology setting. As a result, professional medical organizations have guidelines and recommendations based on the key role of palliative care in oncology. Six elements of the practice sit at the frontier of outpatient oncology palliative care, including the setting and timing of palliative care integration into outpatient oncology, the relationships between primary and specialty palliative care, quality and measurement, research, electronic and technical innovations, and finances. Conclusions: Evidence of clinical and health care system benefits supports the recommendations of professional organizations to integrate palliative care into the routine treatment of patients with advanced cancer. Introduction In fundamental ways, the field of palliative care has developed in the context of oncology, and the majority of both clinical palliative care service and palliative care From the Divisions of General Internal Medicine (MWR), Geriatrics (BC, CR), Hospital Medicine (RB), Department of Medicine, University of California, San Francisco, and the Hospice and Palliative Nurses Association (CD), Pittsburgh, Pennsylvania. Submitted April 15, 2015; accepted June 23, 2015. Address correspondence to Michael W. Rabow, MD, University of California/Mount Zion, 1545 Divisadero Street, Number 313, San Francisco, CA 94143-0320. E-mail: mike.rabow@ucsf.edu Dr Rabow and Ms Dahlin serve as paid consultants to the Center to Advance Palliative Care. Dr Ritchie is a member of the executive committee of the Palliative Care Research Cooperative Group and is president of the American Academy of Hospice and Palliative Medicine. No significant relationships exist between the other authors and the companies/organizations whose products or services may be referenced in this article. October 2015, Vol. 22, No. 4 research involves patients with cancer and is conducted in health care centers focused on providing oncology care. Although the development of palliative care began in the hospital setting for patients during their last days of life, end-of-life hospital care is but a part of the much broader approach to care defined by palliative care. Most patients spend most of their time outside of the hospital, either at home, in other residences such as assisted-living centers, rehabilitation facilities, and in outpatient medical offices. Thus, outpatient or community-based palliative care is recognized as a key frontier in palliative care, and palliative care in the setting of oncology care is the core of that frontier.1 Six elements at the frontier of outpatient oncology palliative care are reviewed, including the (1) setting and timing of integrating palliative care into outpatient oncology, (2) the relationship between primary and specialty palliative care, (3) quality Cancer Control 465 and measurement, (4) research, (5) electronic and technical innovations, and (6) finances. Background Palliative care is defined by the Center to Advance Palliative Care as “specialized medical care for people living with serious illness.”2 This type of care focuses on providing relief from the symptoms and stress of a serious illness, regardless of the diagnosis. The goal of such care is to improve the quality of life for both the patient and his or her family. A team of palliative care specialists, nurses, social workers, and other health care professionals collaborate to provide “an extra layer of support.” Because palliative care is appropriate for any patient, regardless of age or disease stage, it can be provided along with curative treatment.2 The National Cancer Institute defines supportive care as “care given to improve the quality of life of patients who have a serious or life-threatening disease.”3 We conceive of much of traditional oncology supportive care as included in the constellation of services offered as part of the field of palliative care. Others have argued that supportive care is a broader model than palliative care; however, many medical organizations, including the American Cancer Society and the National Cancer Institute, interchangeably use these terms.3-6 The field of palliative care grew out of the tradition of supporting patients facing the symptoms and distresses of illness within healing traditions generally and Western medicine specifically. With the failure of the Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatment trial to improve the care of hospitalized patients at the end of life, the Robert Wood Johnson Foundation, the Soros Foundation, and others supported the development and growth of the field of palliative care.7 In 2006, palliative care was recognized by the American Board of Medical Specialties as a subspecialty of numerous boards of medicine and surgery. The American Academy of Hospice and Palliative Medicine and the Hospice and Palliative Nurses Association have offered a joint annual assembly since 2004, and the technical support provided by the Center to Advance Palliative Care has spurred on the development of an increasing number of inpatient palliative care services.8 The National Palliative Care Research Center (NPCRC) and the National Institute of Nursing Research (NINR)–funded Palliative Care Research Cooperative Group have fostered research in palliative care, with the NPCRC registry having compiled national operational data for inpatient services.9 In addition to the Palliative Care Leadership Center Initiative from the Center to Advance Palliative Care, other major international palliative care education is led by the End-of-Life Nursing Education Consortium.10,11 The California State University Institute for Palliative Care also offers online education for palliative care–re466 Cancer Control lated disciplines.12 Professional medical organizations have offered guidelines and recommendations about the role of palliative care in oncology.13-19 The National Comprehensive Cancer Network (NCCN) is conducting a study of palliative care services among its member organizations (personal communication, Jessica Sugalski, MPPA). Marking the philosophical, clinical, and financial alignment of palliative care and oncology, the American Society of Clinical Oncology (ASCO) launched the first annual Palliative Care in Oncology Symposium in October 2014.20 Benefits The benefits of outpatient palliative care to patients with cancer and their families and health care professionals, in addition to the health care system, have been well documented in a series of studies.21-24 One review catalogued these benefits, noting that much of the research referenced came from studies in oncology.21 The most rigorous study designs include the randomized trials of early palliative care in non–small-cell lung cancer, telephonic palliative care for rural patients in a cancer center, and palliative care across 24 medical oncology clinics in Canada.22-24 Randomized studies demonstrate improved satisfaction, clinical outcomes, mortality, and health care utilization.21 In aligning patient care with individual patient preferences, palliative care helps to prevent unwanted, burdensome, and potentially ineffectual oncology-directed treatments at the end of life.25 The result of this impact on utilization is a demonstrated decrease in the overall cost of care in the setting of improved quality care in outpatient palliative care for patients with cancer.26-29 Palliative care in the oncology setting is helping to align quality and cost.30 Guidelines In light of data demonstrating the benefits of palliative care for patients with cancer, ASCO issued a provisional clinical opinion that “combined standard oncology care and palliative care should be considered early in the course of illness for any patient with metastatic cancer and/or high symptom burden.”13 Thus, ASCO is signaling that palliative care is a routine part of standard oncological care for patients with advanced disease. The American College of Surgeons Commission on Cancer (CoC), which issues basic regulations for oncology centers in the United States, issued standard 2.4 in 2011 that requires these centers to have palliative care services on site or by referral.14 Although the CoC is not explicit about how this standard is enforced, the directive is a powerful statement about the central role of palliative care in standard oncological care. The Institute of Medicine (IOM) also focuses on palliative care and has addressed palliative care in the October 2015, Vol. 22, No. 4 oncology setting.15,16 A palliative focus underlies the recommendation of the IOM and directive of the CoC to oncology centers for universal psychosocial distress screening.17,18 In addition, palliative care is part of the supportive care guidelines updated every year by the NCCN, and palliative care is included as a recommended resource in many of the other NCCN guidelines, including adult cancer pain and ovarian cancer, among others.19,31,32 Prevalence and Characteristics of Care Services Given the fundamental role of palliative care in oncology care and other care programs, relatively little is known about the prevalence and characteristics of outpatient oncology palliative care services on a national scale.4 In 2010, Hui et al33 assessed the institutionally reported prevalence of outpatient palliative care at US National Cancer Institute (NCI)–designated and non–NCI-designated comprehensive cancer centers. They reported that 59% and 22% of all NCI-designated cancer centers and non–NCI-designated centers reported an outpatient palliative care clinic, respectively.33 Rabow et al34 reported on established outpatient palliative care practices in the United States, noting that 80% of patients referred had cancer, and oncologists accounted for 76% of the overall referrers. In 2013, Smith et al35 described the general landscape of outpatient palliative care in the United States and found that the majority of referred patients had cancer. Unpublished data also add to our understanding of outpatient oncologic palliative care practice. In 2014, the NCCN obtained information about the presence of palliative care services from 18 of their 23 member institutions at that time, and all had inpatient palliative care services and 16 (89%) reported offering outpatient palliative care (personal communication, Jessica Sugalski, MPPA). Although national data are limited, some individual states have sought to document the prevalence of outpatient palliative care services. Rabow et al36 studied outpatient palliative care practices affiliated with California hospitals and found that 7% of hospitals within California had affiliated outpatient practices (a proportion not significantly changed compared with a survey conducted 4 years earlier), with nearly one-half of referrals from oncology.37 Many problems exist with these prevalence data. Prevalence estimates for the penetrance of palliative care in oncology centers may be overestimated due to inconsistent definitions of what constitutes outpatient oncology palliative care and the potential inaccuracies of self-reported survey data.38 Except for NCCN data, as yet unpublished, much of the data are not recent.33 Although increasing attention is being paid to outpatient palliative care and many institutions appear to be October 2015, Vol. 22, No. 4 developing services, the evidence for rapid growth is scant. The study of outpatient palliative care affiliated with California hospitals suggested that, although new palliative care programs are developing, established programs are also disappearing to the point that the prevalence of outpatient palliative care may not be changing as many expect.36 Regardless of the specific prevalence figures on local and national scales, the issue of greatest practical importance is how the capacity of outpatient oncologic palliative care compares to the need. ASCO’s recommendation for routine palliative care for all US patients with metastatic cancer or high symptom burden suggests shortages of workforce and clinical capacity.39 With the support of the California HealthCare Foundation, Kerr et al40 assessed the outpatient palliative care capacity in California, including information about the presence of outpatient palliative care services from oncology centers. They estimated a range of need for palliative care based on estimates of the number of all deaths in California and the number of deaths from 7 illnesses, including cancer, thus accounting for the majority of nontraumatic deaths in the state.40 Their estimate suggests that the current capacity for outpatient palliative care (oncological and nononcological) in California is 24% to 37% of the need, with no type of palliative available in nearly 40% of California counties.40 An international study demonstrated that most oncology palliative care specialists felt their institutions were unlikely to expand palliative care services due to financial constraints.41 Although the national prevalence and sustainability of outpatient oncological palliative care programs is not precisely known, interest in the field exists and there are many exciting areas of innovation and growth. Six of these areas are reviewed below. Integration of Care Setting Palliative care specialists seek to integrate palliation within routine oncology care to improve clinical quality.42,43 However, despite widespread calls for integration, no consensus exists about how this is best done. We do not have a clear vision of what constitutes integration or the added value of various elements of integration.4,44 At a basic level, integration is influenced by the setting of palliative care. Although stand-alone palliative care clinics in academic medical centers were some of the first outpatient palliative care services offered to patients with cancer, palliative care services are “embedding” into oncology practices.29 Embedded clinics improve the potential for palliative–oncological integration and collaboration, and they increase convenience for patients able to avail themselves of “onestop” clinical care. Embedded care requires enhanced palliative care competencies for nurses and social Cancer Control 467 workers in these clinics in order to provide interdisciplinary palliative care.45 The precepts of patient-centered care suggest the need for services available to patients with cancer throughout the course of their disease and wherever that patient is living (home, long-term care, rehabilitation facility). Those in outpatient palliative care are recognizing the need for more home-based palliative care services.46,47 Providing “palliative care everywhere,” including in the gaps of care between health care transitions and at night and on weekends, is likely to be necessary to prevent the inefficient utilization of emergency departments and acute care hospitals for preventable symptom-management burdens.48 Although patients who are homebound due to advanced cancer may also be too disabled to benefit from chemotherapy, oncologists may be able to extend the care of homebound patients with orally targeted therapies and home-based palliative care. In fact, home-based palliative care is increasingly recognized as key for patients who are homebound or nearly so and have significant symptomatology but do not qualify for home hospice because of a prognosis longer than 6 months, patient refusal of hospice, or the desire to receive concurrent oncological or other disease-management services (eg, blood transfusions, artificial nutrition) along with palliative care.49 This includes specialty oncology home services that offer monitoring of home infusion of chemotherapy and other fluids.49 Given resource and workforce limitations, innovative palliative care programs are collaborating with hospice organizations or home-based medical and assisted-living services that often have the expertise and capabilities to provide palliative care at home, including in rural and challenging urban settings.49 Timing Outpatient palliative care allows patients with cancer to be seen early in the course of their illness.29 Potential benefits of early outpatient oncology palliative care include aggressive management of adverse events and symptoms, longitudinal psychosocial support for patients and their caregivers, and the facilitation of conversations on the understanding of illness, prognostic understanding, and transitions of care as agreed upon by the health care team. A growing body of literature suggests that early outpatient palliative care is beneficial.22-25,29,50 Three randomized control trials of early, integrated palliative care plus oncological care compared with standard oncological care alone reported improvements in quality of life, depression, and satisfaction among patients with advanced cancer and limited prognoses.22-24,50 Temel et al22 found that 151 patients with newly diagnosed non–small-cell lung cancer had a 2.5-month longer survival rate when they were randomized to early 468 Cancer Control palliative care. Early palliative care has also been associated with reduced health care utilization in studies comparing early palliative care with usual oncology care and late palliative care (ie, > 90 days or < 90 days before death).22,25,29 However, the optimal timing of outpatient palliative care involvement to maximize clinical benefit remains unknown. A recent study randomizing patients with advanced cancer to early in-person outpatient palliative care consultation plus nursing telephonic coaching sessions (within 30–60 days of diagnosis) or late sessions (approximately 4 to 5 months after diagnosis) found no improvements in patient outcomes (eg, quality of life) or health care utilization.23 However, the same study found a 15% improvement in rate of survival for patients receiving early palliative care.23 The results of this study raise questions regarding the optimal timing of palliative care interventions along with the mechanisms that contribute to increased survival rates.23 Further complicating the question of timing is that the benefits of palliative care may differ by cancer type and prognosis. Additional research is needed to guide when, along a patient’s cancer journey, palliative care can have the most reliable and effective impact. In most referral systems, outpatient oncologists play a crucial role in deciding on the need for and timing of palliative care referral.51 Although a significant increase has been seen in the use of palliative care by oncologists overall, data from a national survey suggest that a minority of oncologists frequently refer their patients to pain or palliative care specialists.52,53 Furthermore, when referrals do occur, they tend to be late in the patient’s illness course, typically 30 to 60 days before death.54 Potential explanations for the lack of referrals or late referrals include the misperception by the oncology team members that palliative care means end-of-life care, concern that referral will cause patients to feel defeated, frightened, or lose hope, limited access to services, not knowing the appropriate time to refer, and under-recognition of the patient concerns that might benefit from palliative care consultation. One study found significant differences between the reasons an oncologist referred to palliative care compared with a comprehensive screening system.55 Currently, no national standards for outpatient palliative care referral exist. Identifying reliable markers and developing standardized screening tools to trigger timely outpatient palliative care referral would likely address some barriers to referral and help integrate palliative care into oncology care. Potential indicators could include prognosis using validated prognostic models, index hospitalization for symptom management or loss of function, development of increased symptom burden, referral of patients with cancers with historically poor prognoses and/or high symptom burden, or progression after first-line chemotherapy. October 2015, Vol. 22, No. 4 Primary vs Specialty Palliative Care The future of comprehensive cancer care includes palliative care beginning at diagnosis and, in particular, for advanced disease. Given both the need for and benefits of providing palliation in the oncology setting, as well as the shortages of health care professionals specializing in palliative care, the development of primary palliative care training and competence among oncologists is key. With mandates from ASCO, CoC, NCCN, and IOM for palliative care as part of comprehensive cancer care, outpatient oncology care necessitates that all health care professionals providing oncology care have primary palliative care skills as well as access to palliative care specialists.13-19 All oncologists must learn to understand basic palliative care concepts and to appropriately utilize palliative care as part of comprehensive, patient- and family-focused care. A distinction is emerging between primary palliative care and specialty palliative care within oncology care.56 Although both settings pertain to pain and symptom management and communication, clear delineations exist in skills (Tables 1 and 2).56-59 Primary palliative care addresses relief of symptoms, including basic management of pain and symptoms (eg, depression, anxiety) and communication skills (eg, goals of care inclusive of prognosis, advance care planning, code status). All oncologists can and must learn palliative care skills. Depending on the clinician’s discipline, options exist for primary palliative care education. Nurses, physicians, and social workers may obtain some palliative care competency through their routine education and training; however, this is usually insufficient.60 Oncology physician fellowships offer an overview of palliative care, symptom management, and communication skills. Oncology nurses may receive education in common symptoms for specific cancers and management of those symptoms. Other specialized programs, such as End of Life Nursing Education Consortium and Education in Palliative Care and Endof-life Care for Oncology, focus on the care of patients with cancer.57,61 Another program for psychologists, social workers, and spiritual care professionals is the Advocating for Clinical Excellence: Transdisciplinary Palliative Care Education.62 In real time, the collaboration around individual patients between specialty palliative care teams and oncology teams can create opportunities for cross education. In clinical work together, oncologists can learn primary palliative care concepts and palliative care specialists can learn about primary oncology concepts. Beyond education, new paradigms of primary palliative care in oncology are evolving. One model is an oncology nurse navigator who employs primary palliative care skills to address patient symptom needs, engage patients in their care through advance care planning, provide emotional support, and communicate October 2015, Vol. 22, No. 4 Table 1. — Primary and Specialty Palliative Care in Oncology Care Primary Specialty Basic symptom management of physical and psychological symptoms Cancer-related pain Depression Anxiety Relief of symptoms Complex symptom management of refractory symptoms Complex pain Complex depression Existential distress Communication Cancer prognosis Goals of treatment Communication Conflict management within the circle of patients, families, and health care team Advance care planning Ethics (in particular, medical futility) Adapted from Quill TE, Abernethy AP. Generalist plus specialist palliative care — creating a more sustainable model. N Engl J Med. 2013;368(13):1173-1175. Copyright ©2013 Massachusetts Medical Society. Reprinted with permission from Massachusetts Medical Society. Table 2. — Importance of Palliative Care as Part of Cancer Care Goals Action Primary Palliative Care Recommendations The cancer care team should provide patients and their families with understandable information on cancer prognosis, treatment benefits and harms, palliative care, psychosocial support, and estimates of the total and outof-pocket costs of cancer care. The cancer care team should collaborate with their patients to develop a care plan that reflects their patients’ needs, values, and preferences, and considers palliative care needs and psychosocial support across the cancer care continuum. In the setting of advanced cancer, the cancer care team should provide patients with end-of-life care consistent with their needs, values, and preferences. Professional educational programs for members of the cancer care team should provide comprehensive and formal training in endof-life communication. Specialty Palliative Care Recommendation Members of the cancer care team should coordinate with each other and with primary/ geriatrics and specialist care teams to implement patient care plans and deliver comprehensive, efficient, and patientcentered care. Academic institutions and professional societies should develop interprofessional education programs to train the workforce in team-based cancer care and promote coordination with primary/ geriatrics and specialist care teams. All individuals caring for patients with cancer should have appropriate core competencies. Cancer care delivery organizations should require that the members of the cancer care team have the necessary competencies to deliver high-quality cancer care, as demonstrated through training, certification, or credentials. Republished with permission of National Academies Press. From Institute of Medicine. Delivering High-Quality Cancer Care: Charting a New Course for a System in Crisis. Washington, DC: The National Academies Press; 2013. Permission conveyed through Copyright Clearance Center, Inc. Cancer Control 469 with the oncology team about care needs.63 Other palliative care teams have reported on their primary palliative care education for oncologists.64 Even in the setting of primary palliative care by oncologists, specialist palliative care teams can improve the overall care of patients with cancer.65-72 Specialty palliative care includes the complex management of refractory pain and symptoms, addressing grief and existential distress, and medical futility, as well as more advanced communication such as management of conflict among patients, families, and the health care team. A qualitative review of a small sample of patients with lung cancer who had early intervention specialty palliative care received a range of services, including psycho/spiritual/emotional support, symptom management, advance care planning, and promotion of illness understanding.65 Other studies have demonstrated better symptom control and earlier referral to hospice with specialty palliative care.66,67 By reducing and relieving the stress of care, specialty palliative care saves oncologists time and allows the oncology team to focus on cancer treatment.68,69 Implementation of specialty palliative care within oncology varies and can include palliative care teams that have clinical space in the oncology clinic and are embedded into care delivery, advanced-practice oncology nurses or oncologists who provide oncology care on one day and specialty care on other days, and specialty palliative care clinics within the community.70-72 The relative roles and balance of responsibilities between primary and specialty palliative care teams in the outpatient setting remain to be clarified, but they will need to individualized based on resources, expertise, and access to specialty health care professionals. Integration of primary and specialty palliative care is likely to vary depending on whether the oncology practice is part of an independent practice, hospital, or large health care system. Quality and Metrics The theoretical benefits of outpatient palliative care services or an informal sense of patient and clinician satisfaction were historically sufficient to justify support for services. However, increasingly, outpatient palliative care is being challenged to demonstrate its quality objectively, similar to other fields of medicine. This has ignited a broad conversation about how the quality of outpatient palliative care can be best measured. The American Academy of Hospice and Palliative Medicine and Hospice and Palliative Nurses Association convened a working group to review the existing literature about quality measures in palliative care and to select 10 measures considered to be the highest priority because of their scientific validity and clinical utility to promote across the field.73 The majority of quality metrics prioritized by this initiative pertained to either 470 Cancer Control hospice or inpatient palliative care, because these are the settings in which most of the research has been done.74 An important next step will be to determine how they should be extrapolated to the outpatient palliative care in oncology centers. Similarly, the National Quality Forum formally endorsed 13 quality measures for palliative care in 2012, but the majority of these pertain to patients in the hospital or hospice settings; 1 measure is specifically tailored to measure the quality of outpatient palliative care at oncology centers.75 The Quality Oncology Practice Initiative is an oncologist-led quality assessment program that has promoted a unified set of quality measures for oncology since 2011.76 Its list of metrics includes several that pertain to palliative care, including pain assessment and management, dyspnea assessment, constipation assessment, management of nausea and emesis, provision of hospice and palliative care, and location of death.77 Although evidence for these activities is abstracted from oncologist notes, more complete information about the quality of care that patients with cancer receive could be determined by also examining the work of palliative care specialists. The American College of Surgeons CoC set a new standard to be implemented by 2015 stating that all patients with cancer must be screened at least once for distress and social concerns using a survey instrument (eg, distress thermometer).18,78 The role of palliative care specialists in performing or responding to screening has not yet been determined, but having a robust plan will be critical to respond to the distress identified. Traditionally, quality metrics considered for outpatient palliative care are intended for patients being evaluated by palliative care specialists. However, an even larger — and unanswered — question is how to measure and improve the quality of the primary palliative care that oncology teams provide to patients. In addition to establishing consensus quality metrics for palliative care, methods must be developed to collect and share standardized data across outpatient palliative care programs to benchmark and drive improvement. The Palliative Care Quality Network and the Quality Data Collection Tool provide 2 formats for the prospective collection and comparison of standardized palliative care data.79,80 Prospective data collection makes it possible to obtain patient-reported outcomes in a standardized way, thus allowing for quality improvement efforts that target clinical outcomes (eg, constipation improvement) rather than just processes of care (eg, treatment of constipation). These organizations also provide opportunities for the collaboration of coordinated quality improvement projects across sites that may enable greater gains in quality and safety than any single program could make alone.79,80 Robust measurement, reporting, and quality improvement are important next steps for outpatient palOctober 2015, Vol. 22, No. 4 liative care to help ensure the best care for our patients and to continue to earn the confidence of oncology specialists. Research Recognizing its growing importance, prominent entities such as the IOM, the Patient Centered Outcomes Research Institute, and the National Institutes of Health have recommended that palliative care research become a national priority.81 Many unanswered questions warrant research in outpatient oncology palliative care. The study by Temel et al22 raised a number of key questions, including whether the benefits seen in patients with non–small-cell lung cancer can be generalizable to patients with other cancers, whether their study results can be generalizable to other oncology centers, whether palliative care focused on those with metastatic disease would show the same benefit in if provided to those without metastases, and, of the 7 key elements of palliative care in their intervention, which one would account for the most benefit and which ones are true across different cancers in different settings. Other important research questions in outpatient oncology palliative care include which models of care offer the most effective patient-centered approach to care and which patient and treatment factors contribute most to poor quality of life and how these factors can be modulated to improve patient wellbeing. In 2013, the National Institute of Nursing Research launched the Innovative Questions initiative.82 The goal of this initiative was to initiate a dialogue with the National Institute of Nursing Research stakeholders to identify novel scientific questions. Two of the topic areas were of particular relevance to outpatient oncology palliative care: symptom science and end-of-life/palliative care. A number of these questions directly related to outpatient oncology palliative care research (Table 3).82 Investigators, health care systems, and national organizations are seeking to facilitate research into these questions. The NPCRC promotes palliative care research in an era where many palliative care researchers work in isolation with few other collaborators in their institution.83 The NPCRC also helps to develop junior investigators and supports research that can be rapidly and directly translated into improved clinical practice.83 The Palliative Care Research Cooperative Group focuses on answering research questions that cannot be easily answered at a single site.84 It provides infrastructure for efficient, multisite palliative care research by offering a network of motivated, coordinated, investigators/sites that create capacity for multisite research, as well as methods, tools, and processes that promote the timely and valid conduct of robust cooperative group research.84 These 2 organizations support oncology palliative care research October 2015, Vol. 22, No. 4 Table 3. — Selected Items From the Innovative Questions Initiative How do we overcome barriers in underserved, hard-to-reach populations to implement culturally congruent, patient-, and caregivercentered palliative care strategies? What oncology palliative care interventions/strategies best align with patient and caregiver goals? How do type, intensity, complexity, and fluctuation of symptom burden in oncology patients impact individual and family goals for care? What are the best models for community-based oncology palliative care? What are the strategies for assessing caregiver preparedness and self-care abilities for oncology palliative care early in the illness trajectory? For symptom management at the end of life, what are the best minimally invasive methods to monitor functional status, physiological status, and patient reported outcomes? What electronic data collection methods can be used by health care professionals to monitor, evaluate, and improve palliative/endof-life care? What are the best ways to measure patient reported outcomes using standardized, widely used instruments or common data elements? From National Institute of Nursing Research. The science of compassion: future directions in end-of-life palliative. http://www.ninr.nih.gov/ newsandinformation/iq#.VStKxvldWSr. Accessed September 18, 2015. and seek to facilitate investigator development in research oriented toward oncology palliative care.83,84 Technology To comprehensively care for patients across the continuum of oncology care (in hospitals, clinics, home, and in the community), the future of outpatient palliative care in the oncology setting will depend on technological innovations. Technology is beginning to address barriers of space, time, and the limited clinical availability of specialty expertise, and it will be useful as part of a number of the elements of outpatient palliative care in the oncology setting. Technology can provide patient education and support. Beyond how patients and families use social media, support groups are available online with and without professional facilitation, and smart phone applications and websites exist to assist patients and families with symptom assessment, advance care planning, and family caregiver support.85 The applicability and benefits of a select number of these services have been evaluated.86-88 Telephonic and video health technology can provide effective clinical care for patients unable to travel due to disability or distance.89 Few interventions have been rigorously evaluated.23 Patient outcomes electronically reported are the core to some of these technologies and this will be facilitated by developments in what has been called the “quantified self.”90,91 Key patient documents, including physician orders for Cancer Control 471 life-sustaining treatment paradigms and advance directives, can be stored and accessed online, improving care across the continuum of sites over time. Patient privacy and Health Insurance Portability and Accountability Act concerns must be addressed as clinical care, patient-reported outcomes, and storage of these data and other health documents occur electronically and move online and, thus, outside the physical boundaries of individual medical centers and across state and national lines. The potential for better care from analysis and research using “big data” is profound. Technology can also increase the efficiency of primary palliative care education and consultation. Educational curricula can be shared within computerized health systems and electronic medical records, as well as online. Palliative care specialists can provide e-consults to treating oncologists at a distance. Financing In a fee-for-service system for oncology care, the incentives of individual oncologists and hospitals are aligned with the provision of multiple treatments and services (volume-based payment) without respect to the value of those treatments and services.92 The future of oncology care likely involves the ongoing development and penetration of more global health care budgets, including for oncology care, in which efficiency and value (both clinical benefits and cost) are scrutinized and incentivized. ASCO recommends the early integration of palliative and oncology care as a means to improve quality and value, thus reducing the rising costs of oncology care at the end of life.30,93 Although most research documenting the cost effectiveness of palliative care has been conducted in the inpatient setting, outpatient oncology palliative care has demonstrated its role as part of comprehensive oncology care to improve the efficiency of health care utilization, thereby helping to control costs.22,25,94,95 Palliative care in the outpatient oncology setting will continue to be defined by the historical alignment of quality and cost in today’s environment of health care reform. Conclusions Evidence suggests that palliative care in the outpatient oncology setting is beneficial. As a result, professional organizations have issued guidelines and recommendations about the role of palliative care in oncology.13-19 Palliative care is integral to quality comprehensive care for patients with symptoms from cancer, advanced disease, or other serious illness.58,59 Six areas are at the forefront of outpatient palliative care for patients with cancer, including (1) the setting and timing of palliative care integration into the outpatient setting, (2) the relationship between primary and specialty palliative care, (3) quality and metrics, (4) research, (5) electronic and technology innovations, and (6) health care financ472 Cancer Control ing. Experts in these frontiers have also begun to address the profound challenge of workforce shortages in specialty palliative care. Through the successful integration of oncology and palliative care, all patients with cancer should be able to receive standard, comprehensive, integrated care that allows them to live as long and as well as possible. References 1. Meier DE, Beresford L. Outpatient clinics are a new frontier for palliative care. J Palliat Med. 2008;11(6):823-828. 2. Center to Advance Palliative Care website. https://www.capc.org /about/palliative-care. Accessed September 18, 2015. 3. National Cancer Institute. Dictionary of cancer terms website. http:// www.cancer.gov/publications/dictionaries/cancer-terms?cdrid=46609. Accessed September 22, 2015. 4. Bruera E, Hui D. Conceptual models for integrating palliative care at cancer centers. J Palliat Med. 2012;15(11):1261-1269. 5. Multinational Association of Supportive Care in Cancer website. http://www.mascc.org/about-mascc. Accessed September 18, 2015. 6. American Cancer Society website. http://www.cancer.org/treatment /treatmentsandsideeffects/palliativecare/index. Accessed September 18, 2015. 7. Robert Wood Johnson Foundation. The path to palliative care. Published July 6, 2012. http://www.rwjf.org/en/library/articles-and-news/2012/07/the-pathto-palliative-care.html, accessed 9/22/15. Accessed September 23, 2015. 8. Social Impact Exchange. Center to Advance Palliative care website. http://www.socialimpactexchange.org/organization/center-advance-palliative-care. Accessed September 23, 2015. 9.National Palliative Care Registry website. https://registry.capc.org /cms. Accessed September 18, 2015. 10. Palliative Care Leadership Centers website. https://www.capc.org /palliative-care-leadership-centers. Accessed September 18, 2015. 11. End-of-Life Nursing Education Consortium website. http://www.aacn.nche.edu/elnec. Accessed September 18, 2015. 12. California State University Institute for Palliative Care website. http://csupalliativecare.org. Accessed September 18, 2015. 13. Smith TJ, Temin S, Alesi ER, et al. American Society of Clinical Oncology provisional clinical opinion: the integration of palliative care into standard oncology care. J Clin Oncol. 2012;30(8):880-887. 14. American College of Surgeons. New CoC accreditation standards gain strong support. https://www.facs.org/media/press-releases/2011 /coc-standards0811. Accessed September 18, 2015. 15. Institute of Medicine. Dying in America: Improving Quality and Honoring Individual Preferences Near the End of Life. Washington, DC: National Academies Press; 2015. 16. National Cancer Policy Board, National Research Council. Improving Palliative Care for Cancer. Foley K and Gelband H, Eds. Washington, DC: The National Academies Press, 2001. 17. Institute of Medicine. Cancer Care for the Whole Patient: Meeting Psychosocial Health Needs. Adler NE, Page AEK, eds. National Academies Press, Washington, DC, 2008 18. American College of Surgeons. Commission on Cancer: standard 3.2, psychosocial distress screening. https://www.youtube.com/watch? v=m5PE-i-0jOo. Accessed September 22, 2015. 19. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Palliative Care. v2.2015. http://www.nccn.org/pr ofessionals/physician_gls/PDF/palliative.pdf. Accessed September 21, 2015. 20. Palliative Care in Oncology Symposium website. http://pallonc.org. Accessed September 22, 2015. 21. Rabow M, Kvale E, Barbour L, et al. Moving upstream: a review of the evidence of the impact of outpatient palliative care. J Palliat Med. 2013;16(12):1540-1549. 22. Temel JS, Greer JA, Muzikansky A, et al. Early palliative care for patients with metastatic non-small-cell lung cancer. N Engl J Med. 2010;363(8):733-742. 23. Bakitas MA, Tosteson TD, Li Z, et al. Early versus delayed Initiation of concurrent palliative oncology care: patient outcomes in the ENABLE III randomized controlled trial. J Clin Oncol. 2015;33(13):1438-1445. 24. Zimmermann C, Swami N, Krzyzanowska M, et al. Early palliative care for patients with advanced cancer: a cluster-randomised controlled trial. Lancet. 2014;383(9930):1721-1730. 25. Hui D, Kim SH, Roquemore J, et al. Impact of timing and setting of palliative care referral on quality of end-of-life care in cancer patients. Cancer. 2014;120(11):1743-1749. 26. Brumley R, Enguidanos S, Jamison P, et al. Increased satisfaction with care and lower costs: results of a randomized trial of in-home palliative care. J Am Geriatr Soc. 2007;55(7):993-1000. 27. Greer J, McMahon P, Tramontano A, et al. Effect of early palliative care on health care costs in patients with metastatic NSCLC. J Clin Oncol. October 2015, Vol. 22, No. 4 2012;30(suppl):6004. 28. Jang RW, Krzyzanowska MK, Zimmermann C, et al. Palliative care and the aggressiveness of end-of-life care in patients with advanced pancreatic cancer. J Natl Cancer Inst. 2015;107(3)pii:dju424. 29. Scibetta C, Kerr K, McGuire J, et al. The costs of waiting: implications of the timing of care consultation among a cohort of decedents at a comprehensive cancer center. J Palliat Med. [In press]. 30. Smith TJ, Hillner BE. Bending the cost curve in cancer care. N Engl J Med. 2011;364(21):2060-2065. 31. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Ovarian Cancer, Including Fallopian Tube Cancer and Primary Peritoneal Cancer. v2.2015. http://www.nccn.org/prof essionals/physician_gls/pdf/ovarian.pdf. Accessed September 21, 2015. 32. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Adult Cancer Pain. v2.2015. http://www.nccn.org /professionals/physician_gls/pdf/pain.pdf. Accessed September 21, 2015. 33. Hui D, Elsayem A, De la Cruz M, et al. Availability and integration of palliative care at US cancer centers. JAMA. 2010;303(11):1054-1061. 34. Rabow MW, Smith AK, Braun JL, et al. Outpatient palliative care practices. Arch Intern Med. 2010;170(7):654-655. 35. Smith AK, Thai JN, Bakitas MA, et al. The diverse landscape of palliative care clinics. J Palliat Med. 2013;16(6):661-668. 36. Rabow MW, O’Riordan DL, Pantilat SZ. A statewide survey of adult and pediatric outpatient palliative care services. J Palliat Med. 2014;17(12):1311-1316. 37. Berger GN, O’Riordan DL, Kerr K, Pantilat SZ: Prevalence and characteristics of outpatient palliative care services in California. Arch Intern Med. 2011;171(22):2057-2059. 38. Wentland EJ, Smith KW. Survey Responses: An Evaluation of Their Validity. New York: Academic Press; 1993. 39. Lupu D; American Academy of Hospice and Palliative Medicine Workforce Task Force. Estimate of current hospice and palliative medicine physician workforce shortage. J Pain Symptom Manage. 2010;40(6):899-911. 40. California Healthcare Foundation. Uneven terrain: mapping palliative care need and supply in California. http://www.chcf.org/publi cations/2015/02/palliative-care-data. Accessed September 18, 2015. 41. Davis MP, Strasser F, Cherny N. How well is palliative care integrated into cancer care? A MASCC, ESMO, and EAPC Project. Support Care Cancer. 2015;23(9):2677-2685. 42. Magarotto R, Lunardi G, Coati F, et al. Reduced use of chemotherapy at the end of life in an integrated-care model of oncology and palliative care. Tumori. 2011;97(5):573-577. 43. Partridge AH, Seah DS, King T, et al. Developing a service model that integrates palliative care throughout cancer care: the time is now. J Clin Oncol. 2014;32(29):3330-3336. 44. Hui D, Kim YJ, Park JC, et al. Integration of oncology and palliative care: a systematic review. Oncologist. 2015;20(1):77-83. 45. Center to Advance Palliative Care. IPAL-OP website. ht tps://w w w.capc.org/ipal/ipal-outpatient-palliative-care-ser vices. Accessed September 22, 2015. 46. Kamal AH, Currow DC, Ritchie CS, et al. Community-based palliative care: the natural evolution for palliative care delivery in the U.S. J Pain Symptom Manage. 2013;46(2):254-264. 47. Leff B, Carlson CM, Saliba D, et al. The invisible homebound: setting quality-of-care standards for home-based primary and palliative care. Health Aff (Millwood). 2015;34(1):21-29. 48. Teno JM, Gozalo PL, Bynum JP, et al. Change in end-of-life care for Medicare beneficiaries: site of death, place of care, and health care transitions in 2000, 2005, and 2009. JAMA. 2013;309(5):470-477. 49. Dhiliwal SR, Muckaden M. Impact of specialist home-based palliative care services in a tertiary oncology set up: a prospective non-randomized observational study. Indian J Palliat Care. 2015;21(1):28-34. 50. Bakitas M, Lyons KD, Hegel MT, et al. Effects of a palliative care intervention on clinical outcomes in patients with advanced cancer: the Project ENABLE II randomized controlled trial. JAMA. 2009;302(7):741-749. 51. Charalambous H, Pallis A, Hasan B, et al. Attitudes and referral patterns of lung cancer specialists in Europe to specialized palliative care (SPC) and the practice of early palliative care (EPC). BMC Palliat Care. 2014;13(1):59. 52. Osta BE, Palmer JL, Paraskevopoulos T, et al. Interval between first palliative care consult and death in patients diagnosed with advanced cancer at a comprehensive cancer center. J Palliat Med. 2008;11(1):51-57. 53. Breuer B, Fleishman SB, Cruciani RA, et al. Medical oncologists’ attitudes and practice in cancer pain management: a national survey. J Clin Oncol. 2011;29(36):4769-4775. 54. Hui D, Kim SH, Kwon JH, et al. Access to palliative care among patients treated at a comprehensive cancer center. Oncologist. 2012;17(12):1574-1580. 55. Morita T, Fujimoto K, Namba M, et al. Palliative care needs of cancer outpatients receiving chemotherapy: an audit of a clinical screening project. Support Care Cancer. 2008;16(1):101-107. 56. Quill TE, Abernethy AP. Generalist plus specialist palliative care — creating a more sustainable model. N Engl J Med. 2013;368(13):1173-1175. 57. National Cancer Institute. EPEC-O self-study. http://www.cancer. gov/resources-for/hp/education/epeco. Accessed September 22, 2015. October 2015, Vol. 22, No. 4 58. Institute of Medicine. Delivering High-Quality Cancer Care: Charting a New Course for a System in Crisis. Washington, DC: National Academies Press; 2013. 59. Ferrell BR, Smith TJ, Levit L, et al. Improving the quality of cancer care: implications for palliative care. J Palliat Med. 2014;17(4):393-399. 60. Buss MK, Lessen DS, Sullivan AM, et al. Hematology/oncology fellows’ training in palliative care: results of a national survey. Cancer. 2011;117(18):4304-4311. 61. Coyne P, Paice JA, Ferrell BR, et al. Oncology End-of-Life Nursing Education Consortium training program: improving palliative care in cancer. Oncol Nurs Forum. 2007;34(4):801-807. 62. Otis-Green S, Ferrell B, Spolum M, et al. An overview of the ACE Project-advocating for clinical excellence: transdisciplinary palliative care education. J Cancer Educ. 2009;24(2):120-126. 63. Schenker Y, White D, Rosenzweig M, et al. Care management by oncology nurses to address palliative care needs: a pilot trial to assess feasibility, acceptability, and perceived effectiveness of the CONNECT intervention. J Palliat Med. 2015;18(3):232-240. 64. Reville B, Reifsnyder J, McGuire DB, et al. Education and referral criteria: impact on oncology referrals to palliative care. J Palliat Med. 2013;16(7):786-789. 65. Yoong J, Park ER, Greer JA, et al. Early palliative care in advanced lung cancer: a qualitative study. JAMA Intern Med. 2013;173(4):283-290. 66. Yennurajalingam S, Urbauer DL, Casper KL, et al. Impact of a palliative care consultation team on cancer-related symptoms in advanced cancer patients referred to an outpatient supportive care clinic. J Pain Symptom Manage. 2011;41(1):49-56. 67. Scheffey C, Kestenbaum MG, Wachterman MW, et al. Clinic-based outpatient palliative care before hospice is associated with longer hospice length of service. J Pain Symptom Manage. 2014;48(4):532-539. 68. Muir JC, Daly F, Davis MS, et al. Integrating palliative care into the outpatient, private practice oncology setting. J Pain Symptom Manage. 2010;40(1):126-135. 69. Grant M, Elk R, Ferrell B, et al. Current status of palliative care-clinical implementation, education, and research. CA Cancer J Clin. 2009;59(5):327-335. 70. Partridge A, Seah D, King T, et al. Developing a service model that integrates palliative care throughout cancer care: the time is now. J Clin Oncol. 2014;32(29):3330-3336. 71. Prince-Paul M, Burant CJ, Saltzman JN, et al. The effects of integrating an advanced practice palliative care nurse in a community oncology center: a pilot study. J Support Oncol. 2010;8(1):21-27. 72. Owens DEK, Burson S, Green M, et al. Primary palliative care clinic pilot project demonstrates benefits of a nurse practitioner-directed clinic providing primary and palliative care. Journal of the American Academy of Nurse Practitioners. 2012;24(1):52-58. 73. American Academy of Hospice and Palliative Medicine. Measuring what matters. http://aahpm.org/quality/measuring-what-matters. Accessed September 18, 2015. 74. Dy SM, Kiley KB, Ast K, et al. Measuring what matters: top-ranked quality indicators for hospice and palliative care from the American Academy of Hospice and Palliative Medicine and Hospice and Palliative Nurses Association. J Pain Symptom Manage. 2015;49(4):773-781. 75. National Quality Forum website. https://www.qualityforum.org /QPS/QPSTool.aspx?p=783. Accessed September 18, 2015. 76. ASCO Institute for Quality website. http://qopi.asco.org. Accessed September 18, 2015. 77. Kamal AH, Gradison M, Maguire JM, et al. Quality measures for palliative care in patients with cancer: a systematic review. J Oncol Pract. 2014;10(4):281-287. 78. Wagner LI, Spiegel D, Pearman T. Using the science of psychosocial care to implement the new American College of Surgeons commission on cancer distress screening standard. J Natl Compr Canc Netw. 2013;11(2):214-211. 79. Palliative Care Quality Network website. http://pcqn.org. Accessed September 18, 2015. 80. Quality Data Collection Tool website. http://www.qdact.org/index. html. Accessed September 18, 2015. 81. National Institutes of Health. The Science of Compassion: Future Directions in End-of-Life and Palliative Care Executive Summary. Bethesda, MD; National Institute of Nursing Research; 2004. https://www.ninr.nih. gov/researchandfunding/scienceofcompassion#.VSxJImZElek. Accessed September 18, 2015. 82. National Institute of Nursing Research. The science of compassion: future directions in end-of-life palliative. http://www.ninr.nih.gov /newsandinformation/iq#.VStKxvldWSr. Accessed September 18, 2015. 83. National Palliative Care Research Center website. http://www.npcrc.org. Accessed September 18, 2015. 84. Abernethy AP, Aziz NM, Basch E, et al. A strategy to advance the evidence base in palliative medicine: formation of a palliative care research cooperative group. J Palliat Med. 2010;13(12):1407-1413. 85. Attai DJ, Cowher MS, Al-Hamadani M, et al. Twitter social media is an effective tool for breast cancer patient education and support: patientreported outcomes by survey. J Med Internet Res. 2015;17(7):e188. Cancer Control 473 86. Batenburg A, Das E. Emotional approach coping and the effects of online peer-led support group participation among patients with breast cancer: a longitudinal study. J Med Internet Res. 2014;16(11):e256. 87. Bender JL, Katz J, Ferris LE, et al. What is the role of online support from the perspective of facilitators of face-to-face support groups? A multi-method study of the use of breast cancer online communities. Patient Educ Couns. 2013;93(3):472-479. 88. Hong Y, Peña-Purcell NC, Ory MG. Outcomes of online support and resources for cancer survivors: a systematic literature review. Patient Educ Couns. 2012;86(3):288-296. 89. Ekeland AG, Bowes A, Flottorp S. Effectiveness of telemedicine: a systematic review of reviews. Int J Med Inform. 2010;79(11):736-771. 90. Blum D, Koeberle D, Omlin A, et al. Feasibility and acceptance of electronic monitoring of symptoms and syndromes using a handheld computer in patients with advanced cancer in daily oncology practice. Support Care Cancer. 2014;22(9):2425-2434. 91. Ashley L, Jones H, Forman D, et al. Feasibility test of a UK-scalable electronic system for regular collection of patient-reported outcome measures and linkage with clinical cancer registry data: the electronic Patientreported Outcomes from Cancer Survivors (ePOCS) system. BMC Med Inform Decis Mak. 2011;11:66. 92. Jacobson M, Earle CC, Price M, et al. How Medicare’s payment cuts for cancer chemotherapy drugs changed patterns of treatment. Health Aff (Millwood). 2010;29(7):1391-1399. 93. Smith TJ, Cassel JB. Cost and non-clinical outcomes of palliative care. J Pain Symptom Manage. 2009;38(1):32-44. 94. Morrison RS, Dietrich J, Ladwig S, et al. Palliative care consultation teams cut hospital costs for Medicaid beneficiaries. Health Affairs. 2011;30:454-463. 95. Penrod JD, Deb P, Luhrs C, et al. Cost and utilization outcomes of patients receiving hospital-based palliative care consultation. J Palliat Med. 2006;9:855-860. 474 Cancer Control October 2015, Vol. 22, No. 4 Oncologists should collaborate early with palliative care specialists to help care for AYA patients with cancer. Photo courtesy of Lisa Scholder. Sunlit, 16" × 24". Palliative Care in Adolescents and Young Adults With Cancer Kristine A. Donovan, PhD, Dianne Knight, MD, and Gwendolyn P. Quinn, PhD Background: Cancer survival rates for adolescents and young adults (AYA) have not improved over time relative to children or adults older than 39 years of age. Palliative care is specialized medical care focused on the control of symptoms and relief of suffering with the goal of improving quality of life for the patient and his or her family. To date, the integration of palliative care in AYA patients with cancer remains suboptimal. Methods: We explore the role of palliative care in the continuum of clinical care for AYA patients with cancer. Results: Clinical practice guidelines highlight the need for integrating palliative care for all patients with cancer, including the AYA population. Despite this, a paucity of evidence exists regarding the use of palliative care with AYA patients with cancer. Graduate clinical education represents an opportunity to promote the full inclusion and early integration of palliative care in the care of AYA patients with cancer. Advance care planning is one area where some agreement exists on the unique needs of AYA patients and their families. Conclusions: In general, palliative care is seen as being synonymous with end-of-life care for patients with cancer. However, the emerging trend toward standardizing oncology care to meet the unique medical, psychosocial, and supportive care needs of AYA patients with cancer and their families represents an opportunity for health care professionals to collaborate early with palliative care specialists to control symptoms and relieve suffering in this vulnerable population. Introduction Defined on the basis of age and cancer biology, cancer in adolescents and young adults (AYA) accounts for approximately 6% of all invasive cancers diagnosed on a yearly basis.1 Each year, nearly 70,000 AYA patients in the United States receive a cancer diagnosis.2 The incidence of specific cancers in the AYA population varies From the Department of Supportive Care Medicine (KAD, DK) and the Health Outcomes and Behavior Program (KAD, GPQ), H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida. Submitted June 15, 2015; accepted July 15, 2015. Address correspondence to Kristine A. Donovan, PhD, Moffitt Cancer Center, 12902 Magnolia Drive, Tampa, FL 33612. E-mail: Kristine.Donovan@Moffitt.org No significant relationships exist between the authors and the companies/organizations whose products or services may be referenced in this article. October 2015, Vol. 22, No. 4 across the age span, which is typically defined as between 15 and 39 years.3 In the last 30 years, survival rates for AYA patients have not improved relative to younger and older age groups, and cancer is the leading cause of disease-related death in this population.4-6 This lack of improvement in survival has been attributed to numerous factors, including the unique biology of AYA cancers, limited access to care, delays in diagnosis and treatment, lack of consistency in treatment approaches, patient nonadherence to treatment, and low rates of access to and participation in clinical trials, as well as the unique medical, psychosocial, and supportive care needs of this patient population.7-10 Recent study results suggest that implementation of the Affordable Care Act will positively affect the AYA population; however, whether the applicable regulations will result in improved rates of cancer survival Cancer Control 475 remains to be seen.11,12 Although the continuum of AYA development is different across its age span, many issues have been identified that distinguish AYA from pediatric and adult populations.13-16 Such issues include the transition away from parental dependence toward dependence on peers and social networks, concerns about future family and life plans, limited access to mental health services and social and peer support networks, and disruptions in school or work life with the associated financial challenges.13-15 Although the specific needs of individual AYA patients may vary, as a group, these patients have more in common than not.7 Cancer and its treatment confound the AYA patient’s ability to establish autonomy and make independent decisions about education, employment, relationships, and starting a family.13 In a recent review of palliative care in AYA, Clark and Fasciano17 distinguish AYA patients as a vulnerable population and highlight the chasm between pediatric and adult oncology care where the AYA patient is often lost. This should be alarming to those who care for AYA patients, because research suggests that, as a group, AYA patients tend to experience more complex, more severe, and longer-lasting distress than children or adults with similar diagnoses.18-20 Palliative Care Palliative care is specialized medical care for individuals with serious illnesses. It focuses on control of symptoms and relief of suffering with the goal of improving quality of life (QOL) for patients and their families. Palliative care in cancer is appropriate at any age and at any stage and can be provided along with curative treatment.21 Palliative medicine is a recognized medical specialty in the United States, and current models of palliative care suggest interdisciplinary approaches that include both the primary oncology team and a specialized palliative care team facilitate optimal patient and family care.22,23 In 2012, the American Society of Clinical Oncology issued a provisional clinical opinion calling for the integration of palliative care into standard oncology care for patients with metastatic cancer, high symptom burden, or both.21 This opinion derives from expert consensus and the results of several randomized controlled trials demonstrating the benefits of integrating palliative care into standard oncology care, including a trial of early palliative care in patients with cancer that found an increased survival benefit for those who received palliative care.21,24 Studies also have demonstrated a beneficial effect of early palliative care on QOL in patients with advanced cancer.24-26 By referencing the potentially practicechanging data from these studies, the opinion highlights the increasing relevance of palliative care for the care of all patients with cancer.21 Consistent with the opinion of the American Society of Clinical Oncology, 476 Cancer Control many professional organizations now recommend that patients with cancer be screened for palliative care needs.21,23,27,28 For example, guidelines from the National Comprehensive Cancer Network recommend that all patients with cancer be routinely screened and rescreened at appropriate intervals for palliative care needs.23 The guidelines also recommend early collaboration with palliative medicine specialists to improve QOL.23 Cancer Treatment Despite the focus on symptom control and QOL at any stage of disease or treatment, palliative care teams have historically been more often involved in the care of patients with cancer deemed challenging or at or near the end of life.7,29 The result is that, among oncology care providers, palliative care is generally synonymous with end-of-life care.29 Thus, integrating palliative care into standard oncology care for AYA, like that for pediatric and adult patients with cancer, remains suboptimal.30 To the best of our knowledge, no trial has integrated early palliative care into the care of AYA patients with cancer receiving treatment. Data on symptom burden in AYA patients, especially at the end of life, are limited. Data on AYA patients with cancer are often contained within the adult and pediatric oncology literature, making it difficult to discern the potential effects of palliative care in the vulnerable AYA population.31-33 Nevertheless, data indicate most AYA patients with cancer experience multiple physical and psychological symptoms and often spend their last days of life within an acute care setting, with end-of-life discussions often occurring only when death is imminent.34-36 In one of the few studies to date of symptom burden in AYA patients with advanced cancer, Cohen-Gogo et al35 conducted a retrospective review of the medical records of 45 AYA patients with cancer treated in a specific AYA oncology unit who died as a result of progressive disease during a 2-year period. Diagnoses of sarcoma or a brain tumor predominated and accounted for 78% of diagnoses.35 A total of 40% of patients received palliative chemotherapy during the last month of life.35 The median time between the last cycle of chemotherapy and death was 30 days (range, 2–457 days).35 A total of 24% of patients received radiotherapy in the last month of life, mostly for pain and symptoms related to tumor volume.35 One-third of patients received artificial nutrition during the last week of life.35 The median number of physical symptoms was 4 (range, 1–7).35 Pain and dyspnea were the most common symptoms, particularly among patients with sarcoma, whereas patients with a brain tumor were more likely to experience paralysis, confusion, or coma.35 With respect to psychological symptoms, during the last month of life, all paOctober 2015, Vol. 22, No. 4 tients reported sadness, anxiety, fear of being alone, fear of death, fear of pain, and guilt.35 A total of 77% of patients met with the psychologist on staff at the AYA unit during their initial anticancer treatment; of these patients, 83% continued to receive care from the psychologist during the last month of life.35 In addition, most of the patients admitted to the unit spent more than 2 weeks in the hospital (median, 16 days; range, 0–30 days) during the last month of life.35 Although this study provides only general baseline information about symptoms and patterns of end-oflife care of AYA patients treated in 1 specific AYA unit, it is noteworthy that in most of the patients, end-of-life care continued to be an active period of care.35 Many of the patients experienced substantial physical and psychological symptoms during the last week of life, a finding that supports the need for a well-trained, multidisciplinary palliative care team to collaboratively work with other members of the health care team.35 Data on QOL among AYA patients with advanced cancer are also scarce, either from descriptive observational studies or randomized controlled trials of interventions aimed at enhancing QOL at any point along the disease trajectory. In a systematic review of the literature regarding QOL in AYA patients with cancer, Quinn et al37 identified 35 studies whose outcomes were any psychosocial factors affecting QOL in AYA patients. Most of the studies they reviewed focused on the post-treatment survivorship period and, broadly defined, the psychosocial supportive care needs of AYA cancer “survivors.”37 Only 1 study involved AYA patients with advanced cancer with an eye toward palliation of symptoms — in this case, at the end of life to improve QOL.35 In their review, Quinn et al37 note lack of sufficient QOL measurement tools and lack of evidence-based interventions to improve QOL in AYA at any point in the cancer care trajectory. They conclude by commenting on the unique needs of the AYA population and the emerging trend toward standardizing oncology care for this population (for additional commentary, see Thomas et al38). Palliative care has not been established in standard oncological care for AYA patients with cancer. However, clinical practice guidelines aimed at the care of AYA patients with cancer across the continuum of care — from diagnosis to survivorship, or end of life — have begun to reflect the need for integrated palliative care.15 For example, consistent with the recommendation that oncology care providers collaborate early with palliative medicine specialists to control symptoms and reduce suffering in all patients with cancer, guidelines from the National Comprehensive Cancer Network include several considerations for palliative care.15 The guidelines note that referral to palliative care is appropriate when patients are being treated with curative intent, that palliative care may be initiated at diagnosis (to October 2015, Vol. 22, No. 4 provide the best possible care for patients), and that interdisciplinary members of the palliative care team should have expertise in understanding the psychosocial, emotional, and developmental issues unique to the AYA cancer population.15 The guidelines also specifically note the importance of creating an AYA team that includes palliative care as a means of improving early referrals, research, and patient-centered care.15 Clinical Education The creation of an AYA team that includes a palliative care clinician knowledgeable about the unique needs of AYA patients with cancer highlights a critical fact. As Wiener et al39 note in their review of factors that make the provision of palliative care particularly challenging in AYA, a dearth of clinicians exists, both in medicine and nursing, who have the requisite training and skills in palliative care needed to care for AYA with cancer. Formal palliative care training is limited in the average US medical school curriculum.39-41 Furthermore, training in adolescent medicine in the United States has been described by many graduates of subspecialty medical residency programs as inadequate for clinical practice.39,42,43 Some commentators, who are aware of the chasm that exists between pediatric and adult oncology care, have suggested that the key to appropriate symptom palliation among AYA patients with cancer is to transition the AYA patient to the adult oncology setting.44 Others have emphasized the need for specialized AYA multidisciplinary palliative care teams that can work in both pediatric and adult facilities.45,46 However, the most convincing commentators are those who seek to integrate palliative care into AYA oncological care by offering educational strategies for teaching clinicians about palliative care in the AYA population. To this end, Wiener et al39 have proposed an educational and conceptual model for education aimed at palliative care in the AYA population that acknowledges existing contextual barriers, such as the limited exposure of clinicians-in-training to functional multidisciplinary teams. The model addresses key aspects of clinician development: knowledge (eg, of human development), skills (eg, symptom management, ability to work in teams), and attitudes (eg, about patient dignity).39 Weiner et al39 also advocate for the teaching of palliative care concepts via educational strategies and mentorship with the greatest potential to improve AYA outcomes. With respect to outcomes, their model highlights social outcomes, such as receiving respect, feeling understood, and trusting the system, and physical outcomes.39 Advance Care Planning In comprehensive palliative care, discussions about end-of-life care are paramount, and advance care planCancer Control 477 ning documents are often used to facilitate these discussions.23 Several advance care planning guides are available for adults, including Five Wishes (Age With Dignity, Tallahassee, Florida; https://fivewishesonline.agingwithdignity.org), which is a document that appoints a legal health care decision-maker at the end of life and specifies an individual’s desired therapies for medical care (eg, palliative care). Five Wishes largely focuses on the expressed desire of adults to participate in medical decision-making regarding treatment at the end of life. Researchers have begun to explore whether AYA patients with cancer are similarly motivated to participate in end-of-life discussions and whether they consider these discussions to be beneficial.31,47-52 Evidence does suggest that AYA patients with cancer are interested in having end-of-life discussions and that the patients, their families, and their health care professionals all benefit from such discussions.48,51 Although adult patients with cancer tend to focus on decisionmaking related to their end-of-life care, AYA patients with cancer appear to be more concerned with how they want to be treated and remembered than about decision-making.47 Whether this finding is related to developmental differences (eg, many adolescent patients may depend on their parents to make treatment-related decisions for them) or to a limited understanding of life-support treatment options and the legal aspects of end-of-life care is unclear. Regardless, such findings suggest that advance care planning documents used to facilitate end-of-life discussions with AYA patients should include developmentally appropriate language and terminology and must also reflect AYA patient values and beliefs.47 Thus, advance care planning guides developed for adult patients are not suitable for AYA patients with cancer and their families. Voicing My Choices (Aging With Dignity; www. agingwithdignity.org/voicing-my-choices.php) is an advance care planning guide designed for AYA patients to help them communicate their end-of-life preferences to family, caregivers, and friends. The guide was developed by investigators at the National Cancer Institute and National Institute of Mental Health via iterative formative research using Five Wishes in a population of patients with cancer and pediatric patients infected with HIV.47-49,53 Similar to Five Wishes, Voicing My Choices is designed to facilitate communication between AYA and their families and health care professionals, including care providers from the fields of medicine, nursing, social work, chaplaincy, psychiatry, and psychology. It consists of an introduction followed by 9 sections, each one a separate module, that addresses topics such as how patients wish to be supported so they do not feel alone, who they would want to make their medi478 Cancer Control cal care decisions if they cannot make them on their own, and what they wish their friends and family to know about them. Five Wishes is legally binding. By contrast, Voicing My Choices is designed to be a legacy document that is not legally binding but that fulfills the patient’s final wishes. The effectiveness of Voicing My Choices is predicated on the skills and attitude of the health care professional.53 The guide brings family members or a surrogate decision-maker into the discussion.53 However, it has not been systematically evaluated. Thus, although it is likely to enhance and facilitate discussion between the AYA patient and his or her health care professional, whether the guide serves to facilitate discussion between the AYA patient and his or her family is not yet known. These and other issues are currently being explored in a multi-institutional trial (NCT02108028), so more information about the utility of the guide should be forthcoming. Conclusions Cancer survival rates for adolescents and young adults (AYA) have not improved over time relative to younger and older age groups.4-6 Palliative care is specialized medical care that focuses on control of symptoms and relief of suffering with the goal of improving quality of life for the patient and his or her family. Palliative care is appropriate at any age and at any stage of cancer and can be provided along with curative treatment.21 To date, the integration of palliative care in AYA cancer care remains suboptimal.30 Existing clinical practice guidelines highlight the need for the integration of palliative care into the care of all patients with cancer, including the AYA population.21,23,27,28 Despite this, a paucity of evidence exists regarding the use of palliative care in AYA patients with cancer beyond care in the last few weeks or months of life.35 Graduate clinical education represents an opportunity to promote the full inclusion and early integration of palliative care in the care of AYA patients with cancer.39,42,43 Advance care planning is one area where some agreement exists on the unique needs of AYA patients and their families. Although palliative care is generally still synonymous with end-of-life care in cancer care, the emerging trend toward standardizing oncology care to meet the unique medical, psychosocial, and supportive care needs of AYA patients and their families represents an opportunity for health care professionals to collaborate early with palliative care specialists to control symptoms and reduce suffering in this vulnerable population.15,29,37 References 1. Bleyer A. The adolescent and young adult gap in cancer care and outcome. Curr Probl Pediatr Adolesc Health Care. 2005;35(5):182-217. 2. Report of the Adolescent and Young Adult Oncology Progress Review Group. Closing the gap: research and care imperatives for adolescents and young adults with cancer. Presented at: National Cancer Advisory Board Meeting; September 6, 2006. http://deainfo.nci.nih.gov/advisory/ ncab/139_0906/presentations/AYAO.pdf. Accessed August 21, 2015. October 2015, Vol. 22, No. 4 3. Bleyer A, O’Leary M, Barr R, et al, eds. Cancer Epidemiology in Older Adolescents and Young Adults 15 to 29 Years of Age, Including SEER Incidence and Survival: 1975-2000. NIH Pub. No. 06-5767. Bethesda, MD: National Cancer Institute; 2006. http://seer.cancer.gov/archive/ publications/aya/aya_mono_complete.pdf. Accessed August 21, 2015. 4.Bleyer A, Choi M, Fuller CD, et al. Relative lack of conditional survival improvement in young adults with cancer. Semin Oncol Nurs. 2009;36(5):460-467. 5. Heron M. Deaths: leading causes for 2010. Natl Vital Stat Rep. 201362(6):1-96. 6. American Cancer Society. Cancer Facts & Figures 2015. Atlanta, GA: American Cancer Society; 2015. http://www.cancer.org/acs/groups/ content/@editorial/documents/document/acspc-044552.pdf. Accessed August 27, 2015. 7. Wein S, Pery S, Zer A. Role of palliative care in adolescent and young adult oncology. J Clin Oncol. 2010;28(32):4819-4824. 8. Zebrack B, Mathews-Bradshaw B, Siegel S; LIVESTRONG Young Adult Alliance. Quality cancer care for adolescents and young adults: a position statement. J Clin Oncol. 2010;28(32):4862-4867. 9.Burke ME, Albritton K, Marina N. Challenges in the recruitment of adolescents and young adults to cancer clinical trials. Cancer. 2007;110(11):2385-2393. 10. Shaw PH, Reed DR, Yeager N, et al. Adolescent and young adult (AYA) oncology in the United States: a specialty in its late adolescence. J Pediatr Hematol Oncol. 2015;37(3):161-169. 11. Rosenberg AR, Kroon L, Chen LA, et al. Insurance status and risk of cancer mortality among adolescents and young adults. Cancer. 2015;121(8):1279-1286. 12. Wolfson JA. Piecing together the puzzle of disparities in adolescents and young adults. Cancer Control. 2015;121(8):1168-1171. 13. Zebrack BJ, Block R, Hayes-Lattin B, et al. Psychosocial service use and unmet need among recently diagnosed adolescent and young adult cancer patients. Cancer. 2013;119(1):201-214. 14. Coccia PF, Altman J, Bhatia S, et al. Adolescent and young adult oncology. Clinical practice guidelines in oncology. J Natl Compr Canc Netw. 2012;10(9):1112-1150. 15. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Adolescent and Young Adult (AYA) and Oncology. v2.2015. http://www.nccn.org/professionals/physician_gls/pdf/aya. pdf. Accessed August 21, 2015. 16. Patterson P, McDonald FE, Zebrack B, et al. Emerging issues among adolescent and young adult cancer survivors. Semin Oncol Nurs. 2015;31(1):53-59. 17. Clark JK, Fasciano K. Young adult palliative care: challenges and opportunities. Am J Hosp Palliat Care. 2015;32(1):101-111. 18. Clerici CA, Massimino M, Casanova M, et al. Psychological referral and consultation for adolescents and young adults with cancer treated at pediatric oncology unit. Pediatr Blood Cancer. 2008;51(1):105-109. 19. Sansom-Daly UM, Wakefield CE, Bryant RA, et al. Online groupbased cognitive-behavioural therapy for adolescents and young adults after cancer treatment: a multicenter randomised controlled trial of Recapture Life-AYA. BMC Cancer. 2012;12:339. 20. Zebrack BJ, Corbett V, Embry L, et al. Psychological distress and unsatisfied need for psychosocial support in adolescent and young adult cancer patients during the first year following diagnosis. Psychooncology. 2014;23(11):1267-1275. 21. Smith TJ, Temin S, Alesi ER, et al. American Society of Clinical Oncology provisional clinical opinion: the integration of palliative care into standard oncology care. J Clin Oncol. 2012;30(8):880-887. 22. Levy MH, Back A, Benedetti C, et al. NCCN clinical practice guidelines in oncology: palliative care. J Natl Compr Canc Netw. 2009;7(4):436-473. 23. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Palliative Care. v2.2015. http://www.nccn.org/professionals/physician_gls/PDF/palliative.pdf. Accessed August 21, 2015. 24. Temel JS, Greer JA, Muzikansky A, et al. Early palliative care for patients with metastatic non-small-cell lung cancer. N Engl J Med. 2010;363(8):733-742. 25. Zimmermann C, Swami N, Krzyzanowska M, et al. Early palliative care for patients with advanced cancer: a cluster-randomised controlled trial. Lancet. 2014;383(9930):1721-1730. 26. Bakitas M, Lyons KD, Hegel MT, et al. Effects of a palliative care intervention on clinical outcomes in patients with advanced cancer: the Project ENABLE II randomized controlled trial. JAMA. 2009;302(7):741-749. 27. Edwards MJ. Access to quality cancer care: consensus statement of the American Federation of Clinical Oncologic Societies. Ann Surg Oncol. 1998;5(7):657-659. 28. World Health Organization website. WHO definition of palliative care. Published 2011. http://www.who.int/cancer/palliative/definition/en. Accessed September 4, 2015. 29. Rosenberg AR, Wolfe J. Palliative care for adolescents and young adults with cancer. Clin Oncol Adolesc Young Adults. 2013;3:41-48. 30. Maciasz RM, Arnold RM, Chu E, et al. Does it matter what you call it? A randomized trial of language used to describe palliative care services. Support Care Cancer. 2013;21(12):3411-3419. October 2015, Vol. 22, No. 4 31. Vern-Gross TZ, Lam CG, Graff Z, et al. Patterns of end-of-life care in children with advanced solid tumor malignancies enrolled on a palliative care service. J Pain Symptom Manage. 2015;50(3):305-312. 32. Wolfe J, Orellana L, Ullrich C, et al. Symptoms and distress in children with advanced cancer: Prospective patient-reported outcomes from the PediQUEST Study. J Clin Oncol. 2015;33(17):1928-1935. 33. Kestler SA, LoBiondo-Wood G. Review of symptom experiences in children and adolescents with cancer. Cancer Nurs. 2012;35(2):E31-E49. 34. Bell CJ, Skiles J, Pradhan K, et al. End-of-life experiences in adolescents dying with cancer. Support Care Cancer. 2010;18(7):827-835. 35. Cohen-Gogo S, Marioni G, Laurent S, et al. End of life care in adolescents and young adults with cancer: experience of the adolescent unit of the Institut Gustave Roussy. Eur J Cancer. 2011;47(18):2735-2741. 36. Keim-Malpass J, Erickson JM, Malpass HC. End-of-life care characteristics for young adults with cancer who die in the hospital. J Palliat Med. 2014;17(12):1359-1364. 37. Quinn GP, Gonçalves V, Sehovic I, et al. Quality of life in adolescent and young adult cancer patients: a systematic review of the literature. Patient Relat Outcome Meas. 2015;6:19-51. 38. Thomas DM, Albritton KH, Ferrari A. Adolescent and young adult oncology: an emerging field. J Clin Oncol. 2010;28(32):4781-4782. 39. Wiener L, Weaver MS, Bell CJ, et al. Threading the cloak: palliative care education for care providers of adolescents and young adults with cancer. Clin Oncol Adolesc Young Adults. 2015;5:1-18. 40. Dickinson GE. A quarter century of end-of-life issues in U.S. medical schools. Death Stud. 2002;26(8):635-646. 41. Fraser HC, Kutner JS, Pfeifer MP. Senior medical students’ perceptions of the adequacy of education on end-of-life issues. J Palliat Med. 2001;4(3):337-343. 42. Fox HB, McManus MA, Diaz A, et al. Advancing medical education training in adolescent health. Pediatrics. 2008;121(5):1043-1045. 43. Wender EH, Bijur PE, Boyce WT. Pediatric residency training: ten years after the Task Force report. Pediatrics. 1992;90(6):876-880. 44. Linebarger JS, Ajayi TA, Jones BL. Adolescents and young adults with life-threatening illness: special considerations, transitions in care, and the role of pediatric palliative care. Pediatr Clin North Am. 2014;61(4):785-796. 45. Wilkins KL, D’Agostino N, Penney AM, et al. Supporting adolescents and young adults with cancer through transitions: position statement from the Canadian Task Force on Adolescents and Young Adults with cancer. J Pediatr Hematol Oncol. 2014;36(7):545-551. 46. Edwards J. A model of palliative care for the adolescent with cancer. Int J Palliat Nurs. 2001;7(10):485-488. 47. Wiener L, Ballard E, Brennan T, et al. How I wish to be remembered: the use of an advance care planning document in adolescent and young adult populations. J Palliat Med. 2008;11(10):1309-1313. 48. Wiener L, Zadeh S, Wexler LH, et al. When silence is not golden: engaging adolescents and young adults in discussions around end-of-life care choices. Pediatr Blood Cancer. 2013;60(5):715-718. 49. Wiener L, Zadeh S, Battles H, et al. Allowing adolescents and young adults to plan their end-of-life care. Pediatrics. 2012;130(5):897-905. 50. Lyon ME, Jacobs S, Briggs L, et al. Family-centered advance care planning for teens with cancer. JAMA Pediatr. 2013;167(5):460-467. 51. Walter JK, Rosenberg AR, Feudtner C. Tackling taboo topics: how to have effective advanced care planning discussions with adolescents and young adults with cancer. JAMA Pediatr. 2013;167(5):489-490. 52. Lyon ME, Jacobs S, Briggs L, et al. A longitudinal, randomized, controlled trial of advance care planning for teens with cancer: anxiety, depression, quality of life, advance directives, spirituality. J Adolesc Health. 2014;54(6):710-717. 53. Zadeh S, Pao M, Wiener L. Opening end-of-life discussions: how to introduce Voicing My CHOiCES, an advance care planning guide for adolescents and young adults. Palliat Support Care. 2015;13(3):591-599. Cancer Control 479 In conjunction with palliative care, life expectancy, functional reserve, and the treatment goals of patients with cancer should be continually reviewed and assessed throughout the course of treatment. Photo courtesy of Lisa Scholder. Duality. 18" × 24". Palliative Care in Older Patients With Cancer Lodovico Balducci, MD, Dawn Dolan, PharmD, and Sarah E. Hoffe, MD Background: In general, cancer is a disease of aging, and palliative care is an essential step in the management of cancer in patients who are older. The goal of this article is to review common symptoms of cancer and oncology treatment and their management. Methods: The pertinent medical literature was reviewed. Results: The scope of palliative care includes personalized cancer treatment. This involves choosing treatment options that best fit the needs of each individual patient. Balancing treatment benefits and risks may be challenging in older patients, many of whom have limited life expectancies and decreased functional reserves. The benefits of treatment may diminish, and the risks of such treatment options increase with age. Thus, the first step toward personalized treatment includes determining physiological age, which is best estimated with a comprehensive geriatric assessment. Prevention of common complications, which include neutropenia and mucositis, allows the administration of treatment in full and effective doses. Fatigue is a chronic symptom related to cancer and its treatment and may lead to functional dependence and an increased risk of death. Fatigue might be prevented by daily exercise even during treatment. Other symptoms include pain and feelings of memory loss. Conclusions: The scope of palliative care encompasses more issues that symptom management and, for this reason, palliative care should be provided once the diagnosis of cancer is established. Determining treatment goals is essential to improve the treatment experience. Symptom management is similar in older and young patients, but symptoms in the older population may be associated with more frequent and severe complications. Many options exist to prevent and ameliorate the complications of oncology treatment in the aged. However, more studies should be conducted on the long-term care of older patients who have survived cancer. From the Senior Adult Oncology Program (LB), Pharmacy Service (DD), and Department of Radiation Therapy (SEH), H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida. Submitted May 14, 2015; accepted August 28, 2015. Address correspondence to Lodovico Balducci, MD, Moffitt Cancer Center, 12902 Magnolia Drive, Tampa, FL 33612. E-mail: Lodovico.Balducci@Moffitt.org Dr Balducci is a consultant for and receives honorarium from Teva Pharmaceuticals. He also receives honoraria from Amgen, Astellas Pharma, and Johnson & Johnson. Dr Dolan is a speaker for and receives honorarium from Eisai. No significant relationship exists between Dr Hoffe and the companies/ organizations whose products or services may be referenced in this article. 480 Cancer Control Introduction The goal of palliative care is to improve the disease experience for all patients, irrespective of the outcome.1 In addition to symptom management, the scope of palliative care includes emotional, spiritual, and social needs of patients, their families and friends, and their caregivers. Many palliative care specialists believe that healing is always achievable even when cure is out of reach, because healing involves patients coming to terms with their disease and its symptoms and outcome.2 Palliative care in older patients with cancer is important because integrating palliative care into stanOctober 2015, Vol. 22, No. 4 dard oncological care can improve rates of survival and quality of life as well as reduce the cost of treatment.3 By the year 2020, more than 50% of patients diagnosed with cancer will be aged 70 years and older.4 In addition, older individuals may present different issues unrelated to cancer than their younger counterparts that make palliative care both urgent and challenging.4 Table 1. — Comprehensive Geriatric Assessment Domain Zubrod performance status Activities of daily living Transferring Bathing Grooming Feeding Dressing Going to the bathroom Maintaining continence Instrumental activities of daily living Use of transportation Ability to take medication Use the phone Manage finances Go shopping Provide one’s own meals Polymorbidity Number and severity of medical conditions (excluding cancer) Polypharmacy Number of medications (generally ≥ 5) Redundancy and duplications Medications inappropriate for use in the elderly Risk of drug interactions Lack of necessary medications Emotional status Screening for depression Memory disorders Assessment of memory impairment (common use is Minimental status) Nutrition Presence and risk of malnutrition: Mini Nutritional Assessment Geriatric syndromes Dementia Severe depression Delirium during mild infection or with medications that generally do not cause delirium Repeated falls Continuous dizziness Incontinence Severe osteoporosis Assessing Physiological Age Aging may be defined as a progressive loss of functional reserve in multiple organ systems that eventually leads to disability and death.5 Thus, social support becomes important to the welfare and survival of persons who are elderly when they are unable to accomplish instrumental or basic activities of daily living. Aging is universal but highly individualized, and physiological age is poorly reflected in chronological age.5 Thus, assessing physiological age is paramount to any medical decisions related to older individuals. In the case of cancer management, the questions facing the health care professional may include: • Will the patient die of cancer or with cancer? • Is the patient likely to experience consequences of cancer during his or her lifetime? • Will the patient tolerate the treatment proposed? • What might be the long-term consequences of cancer and its treatment in this patient? The assessment of physiological age is founded on the comprehensive geriatric assessment (CGA), which encompasses medical, emotional, and social domains, because aging is multidimensional and the assessment of several domains is necessary to estimate life expectancy and stress tolerance (Table 1). Several models integrating the CGA with clinical and laboratory parameters allow estimates of tolerances of surgery, cytotoxic chemotherapy, and life expectancy independent of cancer.6-9 This information may be used in negotiating and determining the most appropriate treatment for each patient. Function is assessed in the CGA as a patient’s ability to perform instrumental and basic activities of daily living, because they have prognostic value independent of performance status. No consensus exists concerning the best way to assess polymorbidity and polypharmacy. Several comorbidity scales that account for the number and the severity of comorbid conditions are in use.10 The definition of polypharmacy may include the number of daily drugs, medication redundancy, drugs contraindicated in older individuals, risk of at least a type 1 drug interaction, and the absence of drugs that may be appropriate.11 Depression, including subclinical depression, has been associated with increased risk of mortality if it is left untreated; depression can be assessed with a number of screening tests.12 Many older individuals may be malnourished or at risk of malnutrition; cancer alone is a cause October 2015, Vol. 22, No. 4 Element Assessed Function of malnutrition.13,14 Both forms of malnutrition can be estimated with the Mini Nutritional Assessment, a commonly used tool. Malnutrition is associated with increased mortality rates, surgery risks, and chemotherapy-related complications.15 A number of simple tests may be utilized to screen a patient for memory disorders associated with decreased rates of survival. Of these, the Minimental status, Montreal Cognitive Assessment, and Saint Louis University Mental Status Exam are commonly used tools.16 Assessment of social support status includes available resources, the need for a caregiver, and the caregiver’s availability and effectiveness.17 Geriatric syndromes involve several conditions that become more common with age, although they are not unique of aging, and purport decreased life expectancy and increased risk of functional dependence.18 In addition to estimating cancer-independent life Cancer Control 481 expectancy and risk of treatment-related toxicity, the CGA helps to determine the existence of unsuspected conditions that could interfere with antineoplastic treatment, including various diseases, depression, initial memory disorders, inadequate resources, and inadequate caregiver support.19 Many other instruments have been proposed to assess life expectancy and risk of disability but none has been validated in patients with cancer; however, the “inflammatory index” has been validated in 2 large cohort studies of aging populations.20 Age-related inflammation may be considered chronic and progressive, and the concentration of inflammatory markers, such as cytokines and fibrinolytic products, has been associated with decreased life expectancy and increased risk of disability. The inflammatory index, ie, the sum of the logarithm of interleukin 6 and tumor necrosis factor-α receptor 1 in the circulation, is the most reliable predictor of long-term mortality.20 It is unclear how cancer-related inflammation might affect the inflammatory index. Frailty is a term often used in the geriatric and geriatric oncology literature.21 Frailty can be interpreted in 2 ways. In one case, it is considered a threshold, a critical loss of functional reserve beyond which any stress, even minimal, may have catastrophic consequences. In the other case, it is considered a continuum, a measurement of the degree of aging. Pharmacology of Aging Aging is associated with changes of pharmacokinetics and pharmacodynamics that may affect oncology treatment and the symptoms of cancer (Table 2).22 Reducing the volume of distribution of hydrosoluble agents may be related to reduced total body water, reduced concentration of plasma proteins, and reduced mass of red blood cells, leading to increased area under the curve and toxicity measures of these hydrosoluble medications. It may be mitigated with the correction of anemia. CYP450 hepatic reactions are the most common site of drug interactions. Patients who are elderly are at increased risk for these interactions due to decreased activity of these reactions and polypharmacy. Reduced glomerular filtration rate appears to decrease the excretion of medicaments as well as their active metabolites. For example, although anthracyclines are excreted with bile, some of these anthracyclines give rise to active metabolites (daunorubicinol, idarubicinol) excreted from the kidneys. When the glomerular filtration rate is reduced, compound toxicity, including myelotoxicity, cardiotoxicity, and mucositis, may be increased. Morphine and its congeners are partly metabolized to 6-glucuronides that are active and renally excreted. The increased area under the curve of these metabolites may partly explain the increased effectiveness and toxicity of opioids in older individuals. Aging is associated with increased risk for myelo482 Cancer Control toxicity, mucositis, peripheral neurotoxicity, and cardiotoxicity from cytotoxic agents. Decreased brain volume appears to increase the risk of medication-related delirium, whereas changes in μ:δ opioid receptors may partly explain the increased risk of opioid-related complications.23 These complications can include delirium, constipation, nausea, and vomiting. Setting Treatment Goals Setting treatment goals is the first step of personalized care. The goals of treatment include cure, symptom Table 2. — Select Pharmacological Changes of Aging Parameter Age-Related Change Pharmacokinetics Absorption Uncertain May be reduced due to decreased absorptive surface, decreased gastric secretions and motility, and decreased splanchnic circulation Volume of distribution Decreased for water-soluble agents Increased for lipid-soluble agents Hepatic uptake and metabolism Decreased hepatic uptake due to decreased splanchnic circulation and decreased liver weight Decreased activity of CYP450-dependent reactions No apparent change in glucuronidation reactions Excretion Renal excretion Decreased due to reduction of both glomerular filtration rate and tubular excretion Biliary excretion Unchanged Pharmacodynamics Organs and systems Age-related changes and clinical consequences Hemopoietic system Decreased hemopoietic reserve due to reduced number and function of hemopoietic precursors that may lead to increased risk of myelosuppression by cytotoxic drugs Mucosa Increased susceptibility to cytotoxic agents to decreased stem cells and increased proliferation of cryptal cells Heart Decreased number of cardiomyocytes and increased susceptibility to cardiotoxic drugs Lungs Decreased respiratory reserve Possibly increased susceptibility to cytotoxic agents Brain Decreased brain volume and circulation may lead to increased susceptibility to medication-related damage Possible altered μ:δ receptors may lead to decreased effectiveness and increased toxicity of opioids Peripheral nervous system Increased susceptibility to neurotoxic drugs Kidney Decreased glomerular filtration rate and tubular function may lead to increased susceptibility to nephrotoxic drugs October 2015, Vol. 22, No. 4 management, prolonging survival, and preserving lating to the patient. In general, the primary caregiver quality of life, as well as prolonging active life exof an older patient with cancer is an elderly spouse with pectancy — that is, maintaining functional indepenhealth conditions of his or her own or an adult child dence and preventing functional decline in older who has professional and family responsibilities of his individuals.24 Loss of independence is universally or her own (“Aeneas syndrome”).28 recognized as the worst threat to quality of life in Thus, assessment of the caregiver should be part older patients.25 of the initial evaluation of an older patient undergoing Treatment goals may be at odds with each other. any form of antineoplastic treatment; the health care For example, adjuvant treatment improves the likeliprofessional can be aided by a social worker to evaluhood of cure for cancers of the breast, lung, large bowate the availability, the capability, and the willingness el, and bladder, but it may be associated with intoleraof the caregiver. If the results of such an assessment ble complications. Aromatase inhibitors that represent prove inadequate, then alternative solutions should be the hormonal treatment of choice for hormone-sensisought (eg, involvement of other family members or tive breast cancer can cause severe joint pain and, thus, other caregivers). lead to functional decline, as well as vaginal dryness The caregiver represents a valuable ally for the and dyspareunia. Approximately 15% of older women health care team and is likely to make a difference in decide to forgo a higher likelihood of cancer control the patient’s experience; however, caregivers may be because of these complications.26 Peripheral neuropsusceptible to severe emotional and physical stress that athy is a disabling complication of chemotherapy for may lead to disease, including depression, disability, older individuals and may be caused by drugs comand increased mortality.29 This stress may impact the monly used for adjuvant treatment, such as platinum family of the caregiver as well as compromise the work derivatives and taxanes.22 In most cases, the decisions quality and productivity of the caregiver. By contrast, of forgo chemotherapy is made following exposure to caregiving can be a rewarding emotional and spiritual one of these agents, and, in some cases, the decision experience that inspires self-worth.30 Thus, the health may be made due to compelling reasons prior to initicare professional must train and support the caregiver ating treatment (eg, patients involved in activities that by providing a realistic outline of the likely course of the require manual dexterity). disease and of the difficulties that may emerge, as well Realistic treatment goals should be established as potential solutions, by allowing caregivers to express prior to initiating treatment and revisited during the concerns and show sympathy for them, and by directing course of the treatment. Initial goals should be based caregivers to available resources (eg, support groups). on patient desires, cancer stage, aggressiveness and reIn each encounter with the health care team, the patient sponsiveness to treatment, life expectancy, risk of comand caregiver should be recognized as a dyad held toplications, and available resources (Fig). However, it is gether by the patient’s disease and treatment. important to remember that the values and desires of the patient are paramount in situations in which proManagement longing survival might be achieved at significant perTreatment-Related Complications sonal cost. Complications of radiation therapy and systemic canThe role of the caregiver when he or she is unable cer treatment, including hormonal therapy, cytotoxic or unwilling to provide necessary care is an unresolved and vexing problem.27 Caregivers should provide timely Cancer Patient access to health care profesStage Life expectancy Treatment sionals and to emergency Aggressiveness Treatment tolerance Decisions care, provide assistance at Responsiveness Resources home (eg, symptom management, aid in instrumental activities of daily living that the patient is no longer able Treatment to perform), and emotionally Effectiveness support the patient. In some Toxicity instances, the caregiver may Cost act as the family spokesperson within the health care system and may help manage Fig. — The structure of a medical decision. conflicts within the family reOctober 2015, Vol. 22, No. 4 Cancer Control 483 chemotherapy, and targeted Table 3. — Select Complications of Oncology Treatment in Older Patients therapy, are shown in Table 3. Osteoporosis is a comComplication Causes Possible Prevention/ Treatment plication of aromatase inhibitors that may be used for the Osteoporosis Aromatase inhibitors (breast cancer) Calcium and vitamin D supplementation treatment of breast cancer, Androgen deprivation therapy Bisphosphonates (prostate cancer) and androgen deprivation Denosumab treatment (ADT), which may Myelosuppression Most forms of cytotoxic chemotherapy Dose reductions be used for prostate cancer; Select forms of targeted therapy Prophylactic use of myeloid growth (palbociclib, idelalisib, rituximab, factors (cytotoxic chemotherapy alone) the risk for osteoporosis may ibrutinib, lenalidomide, TKI [chronic Blood transfusions offset the potential benemyeloid leukemia]) Erythropoiesis-stimulating agents fits.31 For example, in an old(cytotoxic chemotherapy alone) er woman prone to falls, aroPlatelet transfusions matase inhibitors may prove Mucositis Cytotoxic chemotherapy (fluorouracil, Prophylactic treatment with supermore hazardous than the methotrexate, anthracycline) saturated solutions of calcium, recurrence of breast cancer. phosphate, and bicarbonates Cytotoxic chemotherapy in combinaSelect patients with prostate tion with radiation Management of mucosal infections cancer may undergo ADT for Select targeted agents (everolimus) Prophylactic positioning of gastric or a small increase of prostatejejunal tube (for radiation therapy and chemotherapy combinations) specific antigen in the serum IV hydration after definitive treatment Diarrhea Cytotoxic chemotherapy (fluorouracil, Antidiarrheal medications of the cancer; however, no methotrexate, irinotecan) Octreotide for severe/resistant cases evidence suggests that this Radiation therapy to the pelvis IV hydration practice is beneficial in the TKIs Steroids (for check-point inhibitors alone) absence of disease (based Anti-EGFR antibodies on imaging results), whereas Immune check-point inhibitors evidence suggests that the Anti-PD and anti-PD-L1 antibodies risk of bone fractures inAnti-CTLA-4 antibodies creases by 50% after 1 year Cardiomyopathy Anthracyclines For anthracyclines, dexrazoxane of ADT.31 Trastuzumab Health care professionCTLA-4 = cytotoxic T-lymphocyte-associated protein 4, EGFR = epidermal growth factor receptor, als must check bone density IV = intravenous, PD = programmed cell death, PD-L1 = programmed cell death ligand 1, TKI = tyrosine at the beginning of a pakinase inhibitor. tient’s treatment; if the value is normal, then it should be maintained with adequate intake of calcium and vitapression is managed with dose reduction and delayed min D. In the presence of osteopenia and osteoporotreatment administration, neutropenia can lead to a desis, then more aggressive treatment with bisphosphocrease in the treatment dose and intensity, which may nates or denosumab may be indicated. Some studies result in reduced effectiveness. Myelopoietic growth have shown that an intensive schedule of intravenous factors (filgrastim and pegfilgrastim) are effective in zoledronic acid may prevent osteoporosis and cancer older individuals.22 Guidelines recommend that parecurrence in postmenopausal women receiving adjutients treated with a chemotherapy dose in an intensity vant treatment with an aromatase inhibitor.32,33 Thus, comparable with cyclophosphamide/doxorubicin/vinthe potential benefit of zoledronic acid should be cristine/prednisone receive prophylactic growth facweighed against its inconvenience, cost, and risk for tors to prevent neutropenic infections instead of treatosteonecrosis of the jaw. ment delays or dose reductions.34,35 Myelodepression from cytotoxic chemotherapy is Several techniques reduce the damage of radiation more prolonged and more severe in older individuals, to normal tissues. The most serious complications of and it may be further aggravated by combination cheradiation damage are seen from combination chemomotherapy/radiotherapy to the chest.22 The risk and therapy/radiotherapy in the management of cancer duration of neutropenia and of neutropenic infections of the upper airways, the esophagus, and the lungs.36 increases with age, and patients aged 65 years or older Severe mucositis may lead to dysphagia, malnutrition, are at increased risk for such complications. In addition and dehydration. Given the limited nutritional and hyto severe and sometimes lethal infections, another risk dric reserve of the elderly, these complications may be of neutropenia includes treatment delay, and both may more severe and lethal for older individuals. The procause decreased therapeutic benefits. When myelodephylactic insertion of a gastric or jejunal tube helps 484 Cancer Control October 2015, Vol. 22, No. 4 provide the patient with nutrition and fluids throughout treatment. In addition, the prophylactic use of hypersaturated calcium solution may assuage the pain of mucositis in the upper digestive tract. Laser and light therapy as well as cryotherapy represent promising treatment options for oral mucositis. When mucosal infections are present, they must be treated, otherwise management consists of temporarily discontinuing oncology treatment and managing pain with opioids. Even in the absence of radiotherapy, mucositis may result from cytotoxic chemotherapy, particularly fluorouracil, methotrexate, and doxorubicin. The management is the same as that recommended for combined chemotherapy/radiotherapy. A keratinocyte-stimulating factor may reduce the risk of mucositis, but it is expensive and cumbersome to administer. Its prophylactic use is not recommended. Diarrhea is a common complication of the same drugs that cause mucositis, tyrosine kinase inhibitors, and radiotherapy to the pelvis, although the use of image-modulated radiation therapy or protons can reduce the risk of diarrhea. In addition to the use of antidiarrheal medication, maintaining proper hydration is paramount.37 In some resistant cases, octreotide may be used. Lactobacillus may be useful in chemotherapy-induced diarrhea and hyperbaric oxygen in radiation proctitis.36 Diarrhea is also a common complication of immune check-point inhibitors, such as ipilimumab, nivolumab, and pembroluzimab.38 In such a case, diarrhea is autoimmune related and may respond to steroids. Many different medications can cause peripheral neuropathy, which is common in patients treated with platinum derivatives (eg, oxaliplatin) and taxanes.22 Peripheral neuropathy may cause symptoms such as loss of sensation, paresthesias, shooting pain, burning pain, weakness, and paresis. One prophylaxis for neuropathy is effective, and that is the timely discontinuance of the offending agents. In some cases, neuropathic pain may respond to high-dose gabapentin or pregabalin, but these drugs may cause somnolence and confusion in older individuals.39 Numbness may be associated with difficulty performing basic and instrumental activities of daily living as well as unstable gait and falls. Cardiomyopathy is a complication of anthracyclines, anthracenediones, and cyclophosphamide in high doses. Anthracycline-related cardiomyopathy may be due to progressive damage of the myocardium by free radicals, and it may be irreversible.40 It is rare prior to a total dose of doxorubicin of 300 mg/m2 (and equivalent doses of other anthracyclines).40 Several approaches may prevent this complication and include concomitant administration of dexrazoxane to prevent the formation of free radicals, administration of doxorubicin as a continuous intravenous infusion, or taken as a low daily dose, and use of liposomal pegylated October 2015, Vol. 22, No. 4 preparations. Dexrazoxane may enhance other treatment complications, including myelosuppression and mucositis. According to the results from 1 study, dexrazoxane may diminish the antineoplastic effect of doxorubicin.40 In general, dexrazoxane is recommended only after at least 2 doses of anthracycline. Administering doxorubicin via continuous infusion may be cumbersome outside of major academic centers, and its effectiveness has been confirmed only in sarcomas and in myeloma.40 Data are insufficient for recommending low daily doses of anthracyclines. Pegylated liposomal doxorubicin may be used in lieu of doxorubicin in patients at high risk of cardiomyopathy, but whether its effectiveness is comparable with doxorubicin is unknown. A different form of cardiac toxicity that is, in most cases, reversible can be caused by the monoclonal antibody trastuzumab, which causes a condition of frozen myocardium; discontinuing the drug reverses the effect.41 Risk of cardiac toxicity with trastuzumab also increases with age, and management consists of suspending the administration of trastuzumab, although the drug may be safely restarted when myocardial function has been restored. Cancer-Related Complications The most common symptom of cancer in older individuals is fatigue.42 Other important manifestations include pain and malnutrition. Fatigue: Fatigue is a condition of exhaustion that does not improve with rest and that interferes with individual functions. The prevalence of fatigue, even in the absence of cancer, increases with age; in older individuals, fatigue is associated with increased risk of functional deficiency and death.43 The mechanisms of fatigue are multiple and not fully understood; they may include increased concentration of inflammatory cytokines in the circulation, tissue hypoxia from anemia, hypogonadism-associated ADT, and sarcopenia, a catabolic condition associated with aging that may be related to a combination of inflammation, malnutrition, and endocrine senescence.44 Radiotherapy and cytotoxic chemotherapy may cause fatigue at least in part through treatment-related anemia. Management of fatigue is controversial and unsatisfactory.42 There is consensus that exercise prevents fatigue, but only if the patient is able to follow an exercise program during cancer treatment.45 Correction of anemia with erythropoiesis-stimulating agents may improve energy level. Although concern exists that erythropoiesis-stimulating agents may stimulate tumor growth, these agents appear safe when the hemoglobin level is not above 12 g/dL and they are used only in patients receiving cytotoxic chemotherapy.44 Blood transfusions may also relieve fatigue but only for a limited time. Cancer Control 485 Pain: The perception of somatic and visceral pain may decrease with age.46 For example, it is not uncommon in older individuals to experience silent myocardial infarction or silent intestinal perforation. Older individuals may have a higher tolerance of pain, because they may consider pain an expected consequence of aging. However, bone pain, in particular, may represent a serious limitation to patient activity and independence. As in any other individual, the assessment of pain of older individuals includes: • Type of pain (eg, dull, aching, burning, spasmodic, shooting) • Localization • Conditions that elicit pain (eg, particular movements, positions) • Grade of pain on a pain scale It is important to note that pain may have some atypical manifestations in older individuals; of these, delirium is one of the most common manifestations. Another age-related challenge is assessing pain in individuals with dementia or in those unable to communicate.46 However, select behavioral manifestations, including grimaces, grunting, screaming, or withdrawal, may suggest the need to manage pain. The management of pain depends on many factors, including the type, location, and intensity of the pain. The most common form of pain is bone pain from metastases. When the pain is localized to a single area, local radiotherapy may suffice. If bone metastases are numerous, then relief of pain in one area may result in the emergence of pain in different areas. Systemic cancer treatment, if effective, represents the most definitive and lasting treatment of pain. Timely use of intravenous bisphosphonates or denosumab may mitigate the symptoms and delay the development of pain. Select radioisotopes are available for the management of bone pain; of these, radium 223 has been shown to relieve pain, with minimal marrow damage, in patients with castrate-resistant prostate cancer.47 Pharmacological management is the mainstay of acute pain control.48,49 Tapering doses of steroids may provide temporary relief of bone pain. Nonsteroidal anti-inflammatory drugs have limited use in older patients due their risks of gastritis and renal insufficiency. Opioids represent the most beneficial class of drugs, but their dose should be slowly titrated because the risk of complications may increase with age.48 Intrathecal administration of opioids may prevent most other types of complications, but this route of administration is cumbersome, expensive, and associated with the risk of spinal infections.48 Administration of opioids should be complemented by a prophylactic bowel regimen to prevent constipation. Methylnaltrexone relieves opioid-related constipation without interfering with analgesic activity.49,50 486 Cancer Control Malnutrition: Malnutrition is common, especially in most advanced cancer stages.51 In patients with cancer of the esophagus and upper digestive tract, malnutrition is often present at diagnosis. Malnutrition is due to a combination of factors, including the consumption of tissues by the cancer, decreased food intake, and absorption.52 Anorexia may be prominent during the last phases of cancer and appears to be mediated by increased concentration of 5HT3 and tryptophan in the brain stem.31 Nutritional support is an essential treatment component for patients with a treatable neoplasm, because malnutrition increases the risk of complications of surgery, radiotherapy, and cytotoxic chemotherapy.52 Enteral nutrition is more effective than parenteral nutrition and is preferred in the presence of a functional gastroenteric tract. Prophylactic insertion of gastric or jejunal tubes in the presence of an impending obstruction of the esophagus and upper digestive tract and the aggressive prevention and management of mucositis, nausea, and vomiting are effective and recommended. The management of anorexia is not well established. The effectiveness of cannabinoids is controversial; use of medroxyprogesterone at a high dose appears to stimulate the appetite, but it may not increase the lean body weight of patients. Anamorelin, which is a synthetic analog of the gastric hormone ghrelin, appears promising in early clinical trials.53 Nutritional support of any type fails to improve the lean body weight of patients whose neoplasm is advanced and untreatable and does not affect the survival of such patients. Hyperalimentation may have a detrimental effect on survival. For these patients, nutritional support is indicated only if it is necessary to maintain quality of life. Survivor Care The survival rates of most cancers have improved for patients of all ages; however, a consensual definition of “survivor” is needed.54 Few data relate to elderly patients who have survived cancer. It is well known that age is a risk factor for developing acute leukemia after cytotoxic chemotherapy, and older cancer survivors are at increased risk of developing a second cancer that may be related or unrelated to the original one. At present, evidence is insufficient to suggest that elderly patients who are survivors of cancer may benefit from additional cancer screening. The main concerns related to elderly cancer survivors include functional dependence and memory disorders.45 Functional dependence may result from a combination of factors, including peripheral neuropathy, sarcopenia, and fatigue. Fatigue appears to play a major role because it is associated with increased risks of functional dependence and mortality among older patients. The most effective treatment of fatigue October 2015, Vol. 22, No. 4 appears to be prevention through regular exercise, nutritional preservation, correction of anemia, and depression management, beginning at the time oncology treatment is initiated. Whether cancer and its treatment are associated with full-blown dementia is controversial; however, cognitive function has been shown to decline as a result of treatment, and this decline may cause distress in patients and their loved ones.45 Distress may be prevented by anticipating the possibility of cognitive decline, but reassuring the patient might include performing a neuropsychological evaluation. The fear of cognitive decline appears to be a major cause of distress, and, thus, normal findings may reassure the patient that decline has not occurred. Conclusions The goal of palliative care is to improve the patient experience; thus, palliative care is most effective when administered together with antineoplastic treatment. After the evaluation of the disease, life expectancy, functional reserve, and treatment goals of the patient should be reviewed and assessed — a process that should be continually reviewed throughout the course of treatment. When feasible, common treatment complications, such as neutropenia and neutropenic infections, should be prevented. Management of fatigue, pain, and malnutrition are paramount to the success of treatment. More information is needed on the elderly cancer survivors, particularly as to whether elderly patients should undergo additional cancer screening and determining the most effective management options for fatigue and cognitive decline in elderly patients. References 1. Ramchandran K, Tribett E, Dietrich B, et al. Integrating palliative care into oncology: a way forward. Cancer Control. 2015;22(4):386-395. 2. Balducci L. Suffering and spirituality: analysis of living experiences. J Pain Symptom Manage. 2011;42(3):479-486. 3. Temel JS, Greer JA, Muzikansky A, et al. Early palliative care for patients with metastatic non-small-cell lung cancer. N Engl J Med. 2010;363(8):733-742. 4. Colloca G, Corsonello A, Marzetti E, et al. Treating cancer in older and oldest old patients. Curr Pharm Des. 2015;21(13):1699-1705. 5. Balducci L. Studying cancer treatment in the elderly patient population. Cancer Control. 2014;21(3):215-220. 6. PACE participants, Audisio RA, Pope D, et al. Shall we operate? Preoperative assessment in elderly cancer patients (PACE) can help. A SIOG surgical task force prospective study. Crit Rev Oncol Hematol. 2008;65(2):156-263. 7. Hurria A, Togawa K, Mohile SG, et al. Predicting chemotherapy toxicity in older adults with cancer: a prospective multicenter study. J Clin Oncol. 2011;29(25):3457-3465. 8. Extermann M, Boler I, Reich RR, et al. Predicting the risk of chemotherapy toxicity in older patients: the Chemotherapy Risk Assessment Scale for High-Age Patients (CRASH) score. Cancer. 2012;118(13):3377-3386. 9. Yourman LC, Lee SJ, Schonberg MA, et al. Prognostic indices for older adults: a systematic review. JAMA. 2012;307(2):182-192. 10. de Groot V, Beckerman H, Lankhorst GJ, Bouter LM. How to measure comorbidity. A critical review of available methods. J Clin Epidemiol. 2003;56(3):221-229. 11. Balducci L, Goetz-Parten D, Steinman MA. Polypharmacy and the management of the older cancer patient. Ann Oncol. 2013;24(suppl 7): vii36-40. 12. Millán-Calenti JC, Maseda A, Rochette S, et al. Mental and psychological conditions, medical comorbidity and functional limitation: October 2015, Vol. 22, No. 4 differential associations in older adults with cognitive impairment, depressive symptoms and co-existence of both. Int J Geriatr Psychiatry. 2011;26(10):1071-1079. 13. Bozzetti F. Evidence-based nutritional support of the elderly cancer patient. Nutrition. 2015;31(4):585-586. 14. Ferrat E, Paillaud E, Laurent M, et al. Predictors of 1-Year Mortality in a Prospective Cohort of Elderly Patients With Cancer. J Gerontol A Biol Sci Med Sci. 2015;70(9):1148-1155. 15.Guigoz Y, Lauque S, Vellas BJ. Identifying the elderly at risk for malnutrition. The Mini Nutritional Assessment. Clin Geriatr Med. 2002;18(4):737-757. 16. Velayudhan L, Ryu SH, Raczek M, et al. Review of brief cognitive tests for patients with suspected dementia. Int Psychogeriatr. 2014;26(8):1247-1262. 17. Bradley PJ. Family caregiver assessment. Essential for effective home health care. J Gerontol Nurs. 2003;29(2):29-36. 18. Carlson C, Merel SE, Yukawa M. Geriatric syndromes and geriatric assessment for the generalist. Med Clin North Am. 2015;99(2):263-279. 19. Hurria A, Wildes T, Blair SL, et al. Senior adult oncology, version 2.2014: clinical practice guidelines in oncology. J Natl Compr Canc Netw. 2014;12(1):82-126. 20. Varadhan R, Yao W, Matteini A, et al. Simple biologically informed inflammatory index of two serum cytokines predicts 10 year all-cause mortality in older adults. J Gerontol A Biol Sci Med Sci. 2014;69(2):165-173. 21. Balducci L. Frailty: a common pathway in aging and cancer. Interdiscip Top Gerontol. 2013;38:61-72. 22. Balducci L. Pharmacology of antineoplastic medications in older cancer patients. Oncology (Williston Park). 2009;23(1):78-85. 23. Takao H, Hayashi N, Ohtomo K. A longitudinal study of brain volume changes in normal aging. Eur J Radiol. 2012;81(10):2801-2804. 24. Laditka JN, Laditka SB. Associations of multiple chronic health conditions with active life expectancy in the United States. Disabil Rehabil. 2015:1-8. 25. Bagshaw SM, Stelfox HT, Johnson JA, et al. Long-term association between frailty and health-related quality of life among survivors of critical illness: a prospective multicenter cohort study. Crit Care Med. 2015;43(5):973-982. 26. Hershman DL, Tsui J, Meyer J, et al. The change from brand-name to generic aromatase inhibitors and hormone therapy adherence for earlystage breast cancer. J Natl Cancer Inst. 2014;106(11). pii:dju319. 27. Kimberlin C, Brushwood D, Allen W, et al. Cancer patient and caregiver experiences: communication and pain management issues. J Pain Symptom Manage. 2004;28(6):566-578. 28. Dominguez LJ. Medicine and the arts. L’incendio di Borgo. Commentary. Acad Med. 2009;84(9):1260-1261. 29. Harding R, Gao W, Jackson D, et al. Comparative analysis of informal caregiver burden in advanced cancer, dementia, and acquired brain injury. J Pain Symptom Manage. 2015;50(4):445-452. 30. Millison MB. Spirituality and the caregiver. Developing an underutilized facet of care. Am J Hosp Care. 1988;5(2):37-44. 31. Balducci L. Bone complications of cancer treatment in the elderly. Oncology (Williston Park). 2010;24(8):741-747. 32. Coleman R, Cameron D, Dodwell D, et al. Adjuvant zoledronic acid in patients with early breast cancer: final efficacy analysis of the AZURE (BIG 01/04) randomised open-label phase 3 trial. Lancet Oncol. 2014;15(9):997-1006. 33. Gnant M, Mlineritsch B, Stoeger H, et al. Zoledronic acid combined with adjuvant endocrine therapy of tamoxifen versus anastrozol plus ovarian function suppression in premenopausal early breast cancer: final analysis of the Austrian Breast and Colorectal Cancer Study Group Trial 12. Ann Oncol. 2015;26(2):313-320. 34. Smith TJ, Khatcheressian J, Lyman GH, et al. 2006 update of recommendations for the use of white blood cell growth factors: an evidencebased clinical practice guideline. J Clin Oncol. 2006;24(19):3187-3205. 35. Crawford J, Armitage J, Balducci L, et al. Myeloid growth factors. J Natl Compr Canc Netw. 2013;11(10):1266-1290. 36. Lalla RV, Bowen J, Barasch A, et al. MASCC/ISOO clinical practice guidelines for the management of mucositis secondary to cancer therapy. Cancer. 2014;120(10):1453-1461. 37. O’Brien BE, Kaklamani VG, Benson AB 3rd. The assessment and management of cancer treatment-related diarrhea. Clin Colorectal Cancer. 2005;4(6):375-381. 38. Cheng R, Cooper A, Kench J, et al. Ipilimumab-induced toxicities and the gastroenterologist. J Gastroenterol Hepatol. 2015;30(4):657-666. 39. Miltenburg NC, Boogerd W. Chemotherapy-induced neuropathy: A comprehensive survey. Cancer Treat Rev. 2014;40(7):872-882. 40. Octavia Y, Tocchetti CG, Gabrielson KL, et al. Doxorubicin-induced cardiomyopathy: from molecular mechanisms to therapeutic strategies. J Mol Cell Cardiol. 2012;52(6):1213-1225. 41. Ayres LR, de Almeida Campos MS, de Oliveira Gozzo T, et al. Trastuzumab induced cardiotoxicity in HER2 positive breast cancer patients attended in a tertiary hospital. Int J Clin Pharm. 2015;37(2):365-372. 42. Browner IS, Smith TJ. Symptom assessment in elderly cancer patients receiving palliative care. Ann Oncol. 2013;24(suppl 7):vii25-29. Cancer Control 487 43. Moreh E, Jacobs JM, Stessman J. Fatigue, function, and mortality in older adults. J Gerontol A Biol Sci Med Sci. 2010;65(8):887-895. 44. Bower JE. Cancer-related fatigue--mechanisms, risk factors, and treatments. Nat Rev Clin Oncol. 2014;11(10):597-609. 45. Balducci L, Fossa SD. Rehabilitation of older cancer patients. Acta Oncol. 2013;52(2):233-238. 46. Gagliese L. Pain and aging: the emergence of a new subfield of pain research. J Pain. 2009;10(4):343-353. 47. Parker C, Nilsson S, Heinrich D, et al. Alpha emitter radium-223 and survival in metastatic prostate cancer. N Engl J Med. 2013;369(3):213-223. 48. American Geriatrics Society Panel on the Pharmacological Management of Persistent Pain in Older Persons. Pharmacological management of persistent pain in older persons. Pain Med. 2009;10(6):1062-1083. 49.Thomas J, Karver S, Cooney GA, et al. Methylnaltrexone for opioid-induced constipation in advanced illness. N Engl J Med. 2008; 358(22):2332-2343. 50. Pergolizzi J, Böger RH, Budd K, et al. Opioids and the management of chronic severe pain in the elderly: consensus statement of an International Expert Panel with focus on the six clinically most often used World Health Organization Step III opioids (buprenorphine, fentanyl, hydromorphone, methadone, morphine, oxycodone). Pain Pract. 2008;8(4):287-313. 51. Cohen S, Nathan JA, Goldberg AL. Muscle wasting in disease: molecular mechanisms and promising therapies. Nat Rev Drug Discov. 2015;14(1):58-74. 52. Nicolini A, Ferrari P, Masoni MC, et al. Malnutrition, anorexia and cachexia in cancer patients: A mini-review on pathogenesis and treatment. Biomed Pharmacother. 2013;67(8):807-817. 53. Zhang H, Garcia JM. Anamorelin hydrochloride for the treatment of cancer-anorexia-cachexia in NSCLC. Expert Opin Pharmacother. 2015;16(8):1245-1253. 54. Santoro A; Balducci L. The complexity of cancer survivorship: a case for personalized medicine. Report of the 2014 Grandangolo conference. J Med Pers. 2014;12:37-43. 488 Cancer Control October 2015, Vol. 22, No. 4 Despite their limited accuracy, clinician prediction of survival, prognostic factors, and prognostic models can help health care professionals estimate survival and inform clinical decision-making. Photo courtesy of Lisa Scholder. Dive Forward. 16" × 24". Prognostication of Survival in Patients With Advanced Cancer: Predicting the Unpredictable? David Hui, MD, MSc Background: Prognosis is a key driver of clinical decision-making. However, available prognostication tools have limited accuracy and variable levels of validation. Methods: Principles of survival prediction and literature on clinician prediction of survival, prognostic factors, and prognostic models were reviewed, with a focus on patients with advanced cancer and a survival rate of a few months or less. Results: The 4 principles of survival prediction are (a) prognostication is a process instead of an event, (b) prognostic factors may evolve over the course of the disease, (c) prognostic accuracy for a given prognostic factor/ tool varies by the definition of accuracy, the patient population, and the time frame of prediction, and (d) the exact timing of death cannot be predicted with certainty. Clinician prediction of survival is the most commonly used approach to formulate prognosis. However, clinicians often overestimate survival rates with the temporal question. Other clinician prediction of survival approaches, such as surprise and probabilistic questions, have higher rates of accuracy. Established prognostic factors in the advanced cancer setting include decreased performance status, delirium, dysphagia, cancer anorexia–cachexia, dyspnea, inflammation, and malnutrition. Novel prognostic factors, such as phase angle, may improve rates of accuracy. Many prognostic models are available, including the Palliative Prognostic Score, the Palliative Prognostic Index, and the Glasgow Prognostic Score. Conclusions: Despite the uncertainty in survival prediction, existing prognostic tools can facilitate clinical decision-making by providing approximated time frames (months, weeks, or days). Future research should focus on clarifying and comparing the rates of accuracy for existing prognostic tools, identifying and validating novel prognostic factors, and linking prognostication to decision-making. From the Department of Palliative Care & Rehabilitation Medicine, MD Anderson Cancer Center, Houston, Texas. Submitted April 15, 2015; accepted July 15, 2015. Address correspondence to David Hui, MD, MSc, Department of Palliative Care & Rehabilitation Medicine, Unit 1414, MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030. E-mail: dhui@mdanderson.org Dr Hui is supported in part by a National Institutes of Health grant (R21CA186000-01A1) and an American Cancer Society Mentored Research Scholar Grant in Applied and Clinical Research (MRSG14-1418-01-CCE). No significant relationship exists between the author and the companies/organizations whose products or services may be referenced in this article. October 2015, Vol. 22, No. 4 Introduction In the last months, weeks, and days of life, patients with advanced cancer may face numerous decisions regarding their personal affairs and health care, many of which depend on how long they will live. For example, patients were less likely to choose chemotherapy at the end of life if they understood that they had a short survival rate.1,2 Similarly for health care professionals, prognosis is a key determinant of clinical decisionmaking because the risk:benefit ratio for many interventions increases as patients approach the last weeks of life. Chemotherapy given to a patient with months Cancer Control 489 of life expectancy may result in tumor response, symptom control, and improved survival; however, the same chemotherapy regimen could cause life-threatening complications if administered to a patient with a poor performance status and a short survival rate.3,4 Moreover, palliative resection, total parenteral nutrition, and insertion of an indwelling pleural catheter are generally appropriate for patients with at least a few months of life expectancy.5 Moreover, hospice eligibility is based on a survival of 6 months or less.6 One study showed that patients were more likely to be referred earlier to hospice if their health care professionals made an accurate prediction of survival.7 Prognosis-based decision-making depends on an ability to accurately estimate survival, which has been a challenge for health care professionals and researchers alike.8 The process of prognostication can be divided into formulation (foreseeing) and communication (foretelling).9,10 Clinicians may formulate prognosis either subjectively (ie, clinician prediction of survival based on intuition) or objectively (ie, actuarial prediction of survival based on prognostic factors and models). Despite the availability of validated prognostic factors and tools, most health care professionals rely on clinician prediction of survival to estimate prognosis because clinician prediction of survival is instantaneous, convenient, and easy to understand. Although clinician prediction of survival often incorporates many known prognostic factors in its determination, each may be assigned a variable weight by different health care professionals. Coupled with variable knowledge, clinical experience, and personality, this results in heterogeneous and often optimistic estimations of life expectancy.11,12 Progress has taken place in the science of prognostication. In this article, some important principles of survival prediction are discussed and the medical literature on clinician prediction of survival, prognostic factors, and prognostic models are reviewed, focusing on patients with advanced cancer with a survival rate of months or less. The implications for clinical decisionmaking and future research directions are also explored. Principles of Prognostication Prognostication is a process instead of an event. A patient’s prognosis may change based on treatment response, development of acute oncological complications (eg, hypercalcemia, spinal cord compression, pulmonary embolism), or competing comorbidities (eg, heart failure). In a study of 352 patients admitted to acute palliative care units who had a median survival of 10 days, the presence of acute symptomatic complications, such as pneumonia, peritonitis, metabolic acidosis, and gastrointestinal bleed, was associated with a higher risk of mortality.13 Patients with a larger number of acute complications also had a shorter survival.13 Thus, it is important for health care 490 Cancer Control Table 1. — Differences in Prognostic Factors Between Patients With Early and Advanced Cancer Variable Early Stage Advanced Stage Prognosis Years, decades Months, weeks, days Inception cohort Cancer-site specific Time of diagnosis Time of consultation All cancer groups Admission/referral to palliative care or hospice Prognostic factors Clinical: stage, laboratory studies Pathological: histology, grade Molecular: gene, microarray Others: treatments, resources Clinical: symptoms, laboratory studies Pathological: NA Molecular: NA Others: treatments, resources Tools Scores: IPI Programs: Adjuvant online Scores: PaP, PPI Programs: PPS Implications Overall outlook on life span Worse prognosis = more intensive cancer treatment New cancer treatment → changes in prognostic factors End-of-life planning Worse prognosis → limit cancer treatment Limited cancer treatment → same prognostic factors IPI = International Prognostic Index, NA = not applicable, PaP = Palliative Prognostic Score, PPI = Palliative Prognostic Index, PPS = Palliative Performance Scale. professionals to revisit prognosis with patients over time. Sentinel events such as cancer diagnosis, disease progression, and hospitalizations should trigger a prognostic discussion. Prognostic factors may vary by the stage of disease. In patients with early stage cancer, prognosis may be driven by cancer biology such as tumor stage, histological grade, and mutation status (Table 1). By contrast, prognostic variables in patients with far advanced disease typically consist of patient-related factors such as performance status, dyspnea, delirium, and cancer anorexia–cachexia.14 In the last days of life, distinctive, bedside physical signs may signal that death is imminent.15,16 Thus, it is important to understand the inception cohort for which the prognostic factors/models were derived and apply the study findings to the appropriate patient population. Terms used to describe the inception cohort in the literature, such as end of life and terminally ill, have been heterogeneously defined.17 A systematic review of the literature clarified that both of these terms refer to patients with “months or less of life expectancy,” which represent the target population of this review.18 Accuracy is an elusive concept in prognostication research. This is because not all prognostic studies consistently report accuracy; and, when reported, October 2015, Vol. 22, No. 4 different investigators may use different metrics to assess accuracy, the accuracy of a prognostic tool varies by patient population and the time frame of prediction, and very few studies examining novel prognostic factors have incorporated a comprehensive list of known prognostic variables for benchmarking and examined reclassification. Discrimination and calibration are 2 key aspects of accuracy.19-21 Discrimination reflects how well a prognostic tool differentiates between patients who died and remained alive by a specific time frame. The Concordance statistic (C-statistic) is often used to examine discrimination, with a value between 0.5 and 1. For a C-statistic to be significant, the 95% confidence interval should not cross 0.5. Because the C-statistic is less sensitive to the addition to a novel prognostic marker to an existing model, reclassification statistics, such as the reclassification calibration statistic, net reclassification improvement, and integrated discrimination improvement should be used to assess the degree of improvement with addition of the new factor.22,23 Calibration represents how well the predicted probability of survival based on a prognostic model matches the actual outcomes. A model is considered to have satisfactory calibration (or goodness-of-fit) if the Hosmer–Lemeshow test gives a P value greater than .05.24,25 Furthermore, the prognostic accuracy could be estimated with sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy. To advance the science of prognostication, the accuracy of existing and novel prognostic markers and models need to be routinely assessed. It may not be possible to prognosticate with 100% accuracy (ie, 100% sensitive and 100% specific). Be- cause death is a probabilistic event, its exact timing cannot be predicted with certainty.26 With disease progression, the likelihood of acute catastrophic complication increases, such as myocardial infarction, pneumonia, and massive bleeding.13 Some patients may survive longer than expected, whereas some may die earlier than expected.27 Thus, health care professionals may want to avoid providing specific numbers when discussing prognosis, because doing so could be misleading.28 Instead, they can acknowledge the uncertainty, guide decision-making by providing general time frames (eg, weeks to months), and advise patients and families to expect the unexpected. If we can make decisions based on approximations, why should we still strive to improve the accuracy of survival prediction? It is because higher accuracy can offer health care professionals greater confidence when communicating with patients and families while also bringing greater clarity to decision-making. Clinician Prediction of Survival Over the last decades, clinician prediction of survival has evolved from the classic, temporal question, “How long do I have?” to the surprise and probabilistic questions. Table 2 highlights the question formats and advantages and disadvantages for each approach. The results of some studies also suggest that how the question about prognosis is asked may impact its rate of accuracy.11,29,30 Temporal Question With the temporal approach, the health care professional is asked the question, “How long will this patient live?” The answer may be provided as a specific Table 2. — Three Approaches to Clinician Prediction of Survival Aspects Temporal Question11,30 Surprise Question29,32 Probabilistic Question11,30 Question How long will this patient live? Would I be surprised if this patient died in (specific time frame)? What is the probability of survival within a specific time frame? Answer Specific time frame Yes or no 0% to 100% (at 10% increments) Definition of an accurate response If estimated time frame was ± 33% of actual survival If “no” answer and patient died within specified time frame or If “yes” answer patient remained alive by specified time frame If answered ≤ 30% probability and patient died within specified time frame or If answered ≥ 70% probability and patient remained alive by specified time frame Accuracy 20%–30% 76%–88% (1-y time frame) 53%–91% (6-mo to 24-h time frames) Advantage Simple, quick Intuitive to provide Understand Simple, quick Intuitive to understand Simple, quick Disadvantage Subjective Unclear if time frame represents the average/median, maximal or minimal May be difficult emotionally to provide a specific number Subjective Time frame dependent The threshold for “surprise” may vary by individual Yes/no answer only Subjective Time frame dependent Probability needs to be interpreted October 2015, Vol. 22, No. 4 Cancer Control 491 time frame (eg, 3 days, 6 months). This is the most commonly used approach to estimate the rate of survival. The answer is relative easy to formulate, communicate, and understand. However, it is often not specified if the answer represents the average, median, maximal, or minimal expected survival, possibly resulting in confusion among health care professionals and patients. Furthermore, some health care professionals may find it psychologically challenging to provide a number and communicate with patients an “expiration date.” Temporal clinician prediction of survival often results in systematically overestimation and has a 20% to 30% rate of accuracy, defined as a predicted survival rate of within ± 33% of actual survival.11,12 Christakis et al31 asked 343 physicians to estimate the survival for 468 patients at the time of hospice referral; the median survival in this cohort was 24 days. A total of 20% of predictions were accurate, 63% were overly optimistic, and 17% were overly pessimistic.31 Female patients, certain medical subspecialties, lack of clinical experience, and a longer duration of the doctor–patient relationship were associated with less accurate predictions.31 Another study that included advanced patients with cancer with a median survival of 12 days found that younger patient age was associated with less accurate, temporal clinician prediction of survival.11 Surprise Question The surprise question poses the following to the health care professional: “Would I be surprised if this patient died in (specific time frame)?” The health care professional can answer “no” if he or she would not be surprised that the patient would die within the predefined period of time and “yes” if he or she felt otherwise. Rather than a number with infinite possibility, as in the temporal question, the answer is binominal (yes or no), which may help to reduce the likelihood of error. However, each health care professional may have a different threshold for “surprise.” Moss et al29 asked 4 oncologists to estimate the 1-year survival rate of 853 patients with cancer using this surprise question. The positive predictive value was 41%, the negative predictive value was 97%, and the accuracy rate was 88%.29 In other words, the surprise question was helpful in identifying patients who would live beyond 1 year but less able to identify patients who were going to die within that time frame. In another study, 42 general practitioners in Italy answered the surprise question for 1-year survival in 231 patients with advanced cancer.32 The positive predictive value was 84%, negative predictive value was 69%, and accuracy was 76%.32 Most recently, Hamano et al33 examined the prognostic accuracy rate of the surprise question in 2,361 patients of Japanese descent who had a median survival of 33 days. With the “7-day” sur492 Cancer Control prise question, the rates of sensitivity and specificity were 85% and 68%, respectively, the positive predictive value was 30%, the negative predictive value was 96%, and the rate of accuracy was 70%.33 By contrast, the “30-day” surprise question had sensitivity and specificity rates of 96% and 37%, respectively, and a positive predictive value of 58%, a negative predictive value of 90%, and a rate of accuracy of 65%.33 The surprise question has been used to identify patients who have a limited survival and, thus, may benefit from various services such as palliative care referral and advance care planning discussions; however, the usefulness of this approach needs to be further validated. One qualitative study examining the use of the surprise question among general practitioners identified some potential concerns, including its subjective nature, difficulty in defining a precise time or event when the health care professional would switch the answer from “yes” to “no,” and disagreement that a “no” answer represents the ideal time for specific actions such as advance care planning discussions.34 Probabilistic Question The third approach to clinician prediction of survival employs the probabilistic question. Instead of the “surprise” wording, it asks the health care professional to state the probability of survival within a specific time frame (at 10% increments from 0% to 100%). The response is considered accurate if the health care professional provided a probability of at least 70% and the patient was alive by the prespecified time frame, or if health care professional provided a probability of up to 30% and death occurred within the time frame. By definition, any probability between 40% and 60% is considered inaccurate because the health care professional expressed ambivalence. The probabilistic approach has a potential advantage over the surprise question because it is not dependent on how “surprise” is interpreted. The probabilistic approach was tested in a cohort of 151 patients with advanced cancer admitted to an acute palliative care unit.11 The median survival was 12 days.11 Both physicians and nurses were asked to provide their estimation of survival from the time of admission related to the following time frames: 24 hours, 48 hours, 1 week, 2 weeks, 1 month, 3 months, and 6 months, and the respective accuracy rates were 71%, 66%, 58%, 56%, 67%, 86%, and 96% for physicians and 91%, 86%, 61%, 53%, 60%, 79%, and 88% for nurses.11 By contrast, the rate of accuracy was significantly lower with the temporal approach (32% for physicians and 18% for nurses).11 Because the same group of health care professionals made predictions in the same cohort of patients, the findings from this study suggested how we pose the question may yield answers with different accuracies.11 October 2015, Vol. 22, No. 4 In another study, physicians and nurses provided daily prognostication in 311 patients from the time of admission to an acute palliative care unit until death or discharge using both the probabilistic approach (24and 48-hour time frames) and the temporal approach.30 The rate of accuracy of the probabilistic approach (40% to 100%) was consistently higher than the temporal approach (10% to 30%) among both professions, although its rate of accuracy decreased as death approached.30 Nurses were more accurate than physicians with the probabilistic approach but not with the temporal approach, suggesting that the time of prognostication, the type of health care professional, and the method of clinician prediction of survival are all determinants of the rate of accuracy.30 Finally, the result highlights the difficulty in identifying patients who are imminently dying even among experienced palliative care physicians and nurses.27,30 Actuarial Estimation of Survival Prognostic factors can generally be classified as disease- and patient-related factors. Patient-related factors have a prominent role in prognostication in the last months or weeks of life. Many tumor-related markers, such as circulating tumor cells, have been shown to have prognostic and predictive utility in patients with metastatic disease; however, their role in patients with only months or weeks of survival need to be further examined.35 For the purpose of this review, the discussion is focused on the most validated prognostic factors and models as well as on several novel prognostic factors. Prognostic Factors Among the multiple symptoms with prognostic significance in the advanced cancer setting are the 4 Ds: decreased performance status, dysphagia and cancer anorexia–cachexia syndrome, delirium, and dyspnea.14,36 Performance status declines in the months before death, with a steeper deterioration in the weeks and days preceding death.37,38 Patients with a lower performance status had a higher likelihood of developing serious adverse events when receiving systematic therapy.3,4 In a study involving 1,655 patients with advanced cancer, Eastern Cooperative Oncology Group performance scale, Palliative Performance Scale (PPS), and Karnofsky Performance Scale were strongly associated with survival, with C-statistics of 0.64, 0.63, and 0.63, respectively.39 A web-based program (Prognostat [University of Victoria, British Columbia, Canada]) is available that provides the historical rates of survival based on PPS, age, sex, and cancer diagnosis; however, further validation is required.40,41 Cancer anorexia–cachexia is another prognostic factor seen in patients with advanced cancer and is associated with elevated inflammatory response and October 2015, Vol. 22, No. 4 poor nutritional status; loss of appetite is a poor prognostic marker.42 Malnutrition assessed by either subjective global assessment or other nutritional indices has also been found to be associated with shortened rates of survival.43-45 A multicenter study showed that a lower baseline body mass index and higher percentage of weight loss were both associated with shorten rates of survival, thus forming the basis for a prognosis-based staging system for cancer anorexia–cachexia.46 Moreover, decreased lean body mass, a hallmark of anorexia–cachexia, has prognostic significance independent of the palliative prognostic score.47 Delirium is another syndrome associated with a shortened rate of survival.13,48 Although delirium is potentially reversible in some patients, many patients with cancer develop irreversible or terminal delirium in the last weeks or days of life.16,49,50 Multiple studies have also confirmed the prognostic role of dyspnea; in particular, patients with dyspnea at rest have a shorter rate of survival than those with episodic dyspnea alone.51,52 Other objective, physiological measures also have prognostic utility. Phase angle, a marker of cellular membrane integrity and hydration, is lower in patients with shorter survival.53,54 A prospective study of 222 patients with advanced cancer and a median survival rate of 106 days found that phase angle was a significant prognostic factor independent of Palliative Prognostic Score (PaP), hypoalbuminemia, and decreased lean body mass.47 Hand-grip strength and maximal expiratory pressure that assess skeletal muscle and respiratory muscle functions, respectively, were also associated with survival in patients with advanced cancer.54 These objective measures show some promise in survival prediction because they are reproducible, noninvasive, easy to use, portable, and inexpensive. However, they need to be further validated before they can be applied in routine practice. Several laboratory abnormalities have prognostic significance in the advanced cancer setting. Markers of inflammatory response, such as elevated C-reactive protein, erythrocyte sedimentation rate, leukocytosis, lymphopenia, and neutrophil:lymphocyte ratio were associated with poor nutritional status and survival.14,44,55 Other markers of decreased survival include hypoalbuminemia (indicative of malnutrition), hypogonadism (associated with decreased lean body mass and performance status), hypercalcemia (often related to tumor progression), hyponatremia, and elevated lactate dehydrogenase.13,56-58 For example, patients with advanced solid tumors who presented with hypercalcemia have a median survival rate of 2 months.59 Prognostic Models Multiple prognostic scoring systems have been developed for patients with advanced cancer. These progCancer Control 493 nostic models typically include many of the established prognostic factors discussed above. Table 3 illustrates several of the well-validated prognostic models in the advanced cancer setting, including PaP, the Palliative Prognostic Index (PPI), and the Glasgow Prognostic Score.55,60-80 General time frames are provided by risk score categories for these 3 prognostic models. The original PaP score does not include delirium, although the addition of this variable to create the Delirium-PaP score results in only slight improvement in its performance.81 A modified version of the Glasgow Prognostic Score assigns no points (instead of 1 point) to hypoalbuminemia alone without an elevated level of C-reactive protein.82 In all of these models, total score is calculated based on the number of prognostic factors (ie, higher score = worse survival) and a probability of survival by a defined time frame is provided based on the risk group category. Other prognostic models have been derived from patients with only months or weeks of survival, including the Objective Prognostic Score, the B12/C-reactive protein Index, the Japan Palliative Oncology StudyPrognostic Index, the Chuang Prognostic Score, the Terminal Cancer Prognostic Score, and the Poor Prognosis Indicator.69,70-73,83-86 Despite the plethora of prognostic models, the one that is the most accurate or superior to clinician prediction of survival alone remains unclear. In a prospective study of 549 patients with advanced cancer and a median survival of 22 days, Maltoni et al87 reported that PaP, Delirium-PaP, PPI, and PPS had respective C-indices of 0.72, 0.73, 0.62, and 0.63 and accuracy rates of 88%, 80%, 72%, and less than 50%. However, the PPI cutoffs used were different from earlier studies.87 In a separate study, Stiel et al88 exam- ined the performance of PPI, PaP, and clinician prediction of survival in 84 patients with cancer. PPI had the highest correlation coefficient with actual rate of survival (0.68), followed by PaP (0.58) and clinician prediction of survival (0.56).88 Clinician prediction of survival was examined as a categorical variable instead of as a continuous variable.88 In a large prospective study in Japan, Baba et al89 examined the feasibility and accuracy of PaP, Delirium PaP, and PPI in 2,361 patients in both hospital and home settings. Although PPI was completed more often, PaP and Delirium PaP scores had higher rates of accuracy than PPI for predicting 21-day survival rates (C-statistic, 0.79–0.89 vs 0.75–0.84; P < .05) and 42-day survival rates (C-statistic, 0.81–0.88 vs 0.75–0.85; P < .05).89 The change in a prognostic score may also be useful for predicting survival, with the understanding that patients who deteriorate often have a worse prognostic score over time and vice versa.84,90 In a study of 2,392 patients with advanced cancer, Kao et al90 found that the median PPI increased from 6 on day 1 to 7 on day 8 (P < .001). The median survival rate was 53 days with an improvement in PPI score, 36 days with a stable PPI score, and 22 days with PPI deterioration over the 1-week period.90 The C-statistic for 30-day survival was 0.63 for a baseline PPI score, 0.64 for a PPI change score, and 0.71 for the combined baseline and change in PPI.90 When only patients with a higher baseline PPI (ie, > 6) were included, the C-statistics for 30-, 60-, and 90-day survival rates were 0.66, 0.64, and 0.63 for the baseline PPI, respectively, and 0.72, 0.76, and 0.79 for the magnitude of PPI change between baseline and day 8.74 The corresponding accuracy rates were 61%, 57%, and 55% for baseline PPI, and 71%, 79%, and 83% for PPI change, respectively.74 By definition, the Table 3. — Prognostic Models for Patients With Advanced Cancer Model Variable Scoring Survival Interpretation Palliative Prognostic Score60-63 Clinician prediction of survival (0–8.5) Karnofsky performance scale ≥ 50% (2.5) Anorexia (1.5) Dyspnea (1) Leukocytosis (0–1.5) Lymphopenia (0–2.5) Total score 0–17.5 points Higher score = worse survival Risk group A (0–5.5 points): months of survival Risk group B (5.6–11 points): weeks of survival Risk group C (11.1–17.5 points): days of survival Palliative Prognostic Index74-80 Palliative performance scale (0–4) Delirium (considered absent if caused by a single medication and potentially reversible) (4) Dyspnea at rest (3.5) Oral intake (0–2.5) Edema (1) Total score 0–15 points Higher score = worse survival Risk group A (0–4 points): months of survival Risk group B (4.1–6 points): weeks of survival Risk group C (6.1–15 points): days of survival Glasgow Prognostic Score55,64-68 Albumin < 35 g/L (1) C-reactive protein > 10 mg/L (1) Total score 0–2 Higher score = worse survival Score = 0: months to years of survival Score = 1: months of survival Score = 2: weeks to months of survival 494 Cancer Control October 2015, Vol. 22, No. 4 change score could only be used in patients who remained alive 1 week after the initial assessment, which may limit its utility to a certain extent; nevertheless, a change in PPI over only 1 week had prognostic value.74 Further studies are needed to examine the prognostic utility of change over different time periods and with different prognostic scores. Web-Based Programs Few web-based prognostication programs are available for patients with advanced cancer. Thus, webbased programs should be developed to facilitate the use of validated prognostic models for clinical decision-making. Existing programs (eg, Prognostat) have limited use.40,41 Future Directions Communicating Prognosis Although it is beyond the scope of this review, how health care professionals discuss prognoses with patients and families also warrants further research. Should we give the maximum, minimum, and/or median survival times (as in the temporal approach), the probability of survival at various time points (as in the probabilistic question and prognostic models), or general time frames (eg, days, weeks, months)? Given that the rate of accuracy is below 80% for a vast majority of prognostic tools, perhaps communicating prognoses using general time frames would provide adequate information for decision-making while not being misleading. Ultimately, research studies are needed in this area. Accuracy Reporting The discrimination and calibration of prognostic markers (eg, clinician prediction of survival, prognostic factors and models) should be consistently reported using standardized, predefined, and practical time frames (eg, 1 week, 1 month, 2 months, 3 months, 6 months) that would allow comparison across studies. Clinician Prediction of Survival How we pose the questions matters. In addition, the rate of accuracy of clinician prediction of survival varies by clinician factors, patient characteristics, and time frame of prediction. To date, the surprise question has only been tested with the 1-year, 30-day, and 7-day survival time frames in advanced patients with cancer.33 The probabilistic question has been examined with the time frames of 24 hours, 48 hours, 1 week, 2 weeks, 1 month, 3 months, and 6 months; however, this was only conducted in patients admitted to palliative care units.11,30 Thus, these rates of clinician prediction of survival must be further validated. A direct comparison of the accuracy rate of these approaches is also needed. Prognostic Factors/Models Existing: Validation of existing prognostic factors/ models is important. Furthermore, comparison of the performance of these prognostic tools is warranted to help health care professionals identify the most appropriate model(s) for clinical practice. Novel: A better understanding of the physiological and pathological processes in patients with cancer may help develop novel prognostic markers. Research studies of novel prognostic markers should aim at improving the rate of accuracy of established prognostic model; thus, reclassification statistics should be consistently reported. The role of serial prognostication should also be further examined. Diagnosis of Impending Death Recognition that a patient has entered the last days of life presents a unique area for research. Instead of a prognostic question, this may be a diagnostic issue because the process of dying is irreversible. The results of some studies have suggested that several bedside clinical signs have very high specificity rates for impending death; however, further validation is required.15,16 October 2015, Vol. 22, No. 4 Prognostic Factors Clinical Decision-Making: More studies are needed to examine how prognostic tools can be used to guide clinical decisions, such as palliative care referral or chemotherapy discontinuation. Clinical Trials: In addition to clinical decisionmaking, prognostic factors and models may be incorporated as eligibility criteria or stratification factors in clinical trials of oncology treatment. Currently, performance status is commonly included. Some prognostic markers may have both predictive and prognostic utility. Conclusions Clinician prediction of survival remains the most commonly used approach to formulating a prognosis, a fact that can be attributed to convenience (clinician prediction of survival already incorporates many existing prognostic factors), and few studies have demonstrated that use of prognostic models can significantly improve rates of accuracy. However, health care professionals often overestimate survival with the temporal question, and other clinician prediction of survival approaches, such as the surprise question and the probabilistic question, may estimate survival with a defined time frame and have moderate to high accuracies. 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Prognostic significance of a systemic inflammatory response in patients receiving first-line palliative chemotherapy for recurred or metastatic gastric cancer. BMC Cancer. 2011;11:489. 66. Jeong JH, Lim SM, Yun JY, et al. Comparison of two inflammationbased prognostic scores in patients with unresectable advanced gastric cancer. Oncology. 2012;83(5):292-299. 67. Jiang AG, Lu HY. The Glasgow prognostic score as a prognostic factor in patients with advanced non-small cell lung cancer treated with cisplatin-based first-line chemotherapy. J Chemother. 2015;27(1):35-39. 68. Miura T, Matsumoto Y, Hama T, et al. Glasgow prognostic score predicts prognosis for cancer patients in palliative settings: a subanalysis of the Japan-prognostic assessment tools validation (J-ProVal) study. Support Care Cancer. 2015;23(11):3149-3156. 69. Yoon SJ, Jung JG, Kim JS, et al. Retrospective assessment of objective prognostic score in terminally ill Korean patients with cancer. Am J Hosp Palliat Care. 2014;31(4):435-440. 70. Chuang RB, Hu WY, Chiu TY, et al. Prediction of survival in terminal cancer patients in Taiwan: constructing a prognostic scale. J Pain Symptom Manage. 2004;28(2):115-122. 71. Al-Zahrani AS, El-Kashif AT, Mohammad AA, et al. Prediction of in-hospital mortality of patients with advanced cancer using the Chuang Prognostic Score. Am J Hosp Palliat Care. 2013;30(7):707-711. 72. Yun YH, Heo DS, Heo BY, et al. Development of terminal cancer prognostic score as an index in terminally ill cancer patients. Oncol Rep. 2001;8(4):795-800. 73. Bruera E, Miller MJ, Kuehn N, et al. Estimate of survival of patients admitted to a palliative care unit: a prospective study. J Pain Symptom Manage. 1992;7(2):82-86. 74. Hung CY, Wang HM, Kao CY, et al. Magnitude of score change for the palliative prognostic index for survival prediction in patients with poor prognostic terminal cancer. Support Care Cancer. 2014;22(10):2725-2731. 75. Morita T, Tsunoda J, Inoue S, et al. The Palliative Prognostic Index: a scoring system for survival prediction of terminally ill cancer patients. Support Care Cancer. 1999;7(3):128-133. 76. Sonoda H, Yamaguchi T, Matsumoto M, et al. Validation of the palliative prognostic index and palliative prognostic score in a palliative care consultation team setting for patients with advanced cancers in an acute care hospital in Japan. Am J Hosp Palliat Care. 2014;31(7):730-734. 77. Stone CA, Tiernan E, Dooley BA. Prospective validation of the palliative prognostic index in patients with cancer. J Pain Symptom Manage. 2008;35(6):617-622. 78. Alshemmari S, Ezzat H, Samir Z, et al. The palliative prognostic index for the prediction of survival and in-hospital mortality of patients with advanced cancer in Kuwait. J Palliat Med. 2012;15(2):200-204. 79. Subramaniam S, Thorns A, Ridout M, et al. Accuracy of prognosis prediction by PPI in hospice inpatients with cancer: a multi-centre prospective study. BMJ Support Palliat Care. 2013;3(3):324-329. 80. Hamano J, Kizawa Y, Maeno T, et al. Prospective clarification of the utility of the palliative prognostic index for patients with advanced cancer in the home care setting. Am J Hosp Palliat Care. 2014;31(8):820-824. October 2015, Vol. 22, No. 4 81. Scarpi E, Maltoni M, Miceli R, et al. Survival prediction for terminally ill cancer patients: revision of the palliative prognostic score with incorporation of delirium. Oncologist. 2011;16(12):1793-1799. 82. McMillan DC, Crozier JE, Canna K, et al. Evaluation of an inflammation-based prognostic score (GPS) in patients undergoing resection for colon and rectal cancer. Int J Colorectal Dis. 2007;22(8):881-886. 83. Suh SY, Choi YS, Shim JY, et al. Construction of a new, objective prognostic score for terminally ill cancer patients: a multicenter study. Support Care Cancer. 2010;18(2):151-157. 84. Kelly L, White S, Stone PC. The B12/CRP index as a simple prognostic indicator in patients with advanced cancer: a confirmatory study. Ann Oncol. 2007;18(8):1395-1399. 85. Geissbuhler P, Mermillod B, Rapin CH. Elevated serum vitamin B12 levels associated with CRP as a predictive factor of mortality in palliative care cancer patients: a prospective study over five years. J Pain Symptom Manage. 2000;20(2):93-103. 86. Hyodo I, Morita T, Adachi I, et al. Development of a predicting tool for survival of terminally ill cancer patients. Jpn J Clin Oncol. 2010;40(5):442-448. 87. Maltoni M, Scarpi E, Pittureri C, et al. Prospective comparison of prognostic scores in palliative care cancer populations. Oncologist. 2012;17(3):446-454. 88. Stiel S, Bertram L, Neuhaus S, et al. Evaluation and comparison of two prognostic scores and the physicians’ estimate of survival in terminally ill patients. Support Care Cancer. 2010;18(1):43-49. 89. Baba M, Maeda I, Morita T, et al. Survival prediction for advanced cancer patients in the real world: a comparison of the Palliative Prognostic Score, Delirium-Palliative Prognostic Score, Palliative Prognostic Index and modified Prognosis in Palliative Care Study predictor model. Eur J Cancer. 2015;51(12):1618-1629. 90. Kao CY, Hung YS, Wang HM, et al. Combination of initial palliative prognostic index and score change provides a better prognostic value for terminally ill cancer patients: a six-year observational cohort study. J Pain Symptom Manage. 2014;48(5):804-814. Cancer Control 497 Pathology Perspective Is DOG1 Immunoreactivity Specific to Gastrointestinal Stromal Tumor? William Swalchick, MA, Rania Shamekh, MD, and Marilyn M. Bui, MD, PhD Background: DOG1 is a novel gene on gastrointestinal stromal tumors (GISTs) that encodes the chloride channel protein anoctamin 1, also known as discovered on GIST-1 (DOG1) protein. DOG1 antibodies are a sensitive and specific marker against GIST positive for CD117 and CD34 and negative for CD117 and CD34. DOG1 is also independent of KIT or PDGFRA mutation status and considered specific for GIST when it was first discovered in 2004. Methods: The previous 10 years of literature was searched for articles relating to DOG1. We critically reviewed 12 studies that showed DOG1 was positive in 250 cases of 2,360 tested non-GIST neoplasms (10.6%) at different anatomical sites using monoclonal, polyclonal, or nonspecified antibodies. Criteria for positivity varied between the studies. Results: Monoclonal and polyclonal DOG1 antibodies were reactive in various different non-GIST tumor types spanning 9 organ systems in addition to normal salivary and pancreatic tissues. The tumors included were renal oncocytoma (100%), renal cell carcinoma chromophobe type (86%), solid pseudopapillary neoplasm of the pancreas (51%), neoplastic salivary tissue (17%), synovial sarcoma (15%), leiomyoma (10%), pancreatic adenocarcinoma (7%), and leiomyosarcoma (4%). Conclusions: By contrast to the original concept that DOG1 antibodies are specific to GIST neoplasms, the studies reviewed showed that the data suggest DOG1 positivity in select non-GIST tumors. Only in the appropriate clinical and pathological context is DOG1 positivity specific and helpful in the diagnosis of GIST. Introduction A 58-year-old woman who did not smoke was found to have a 1-cm left upper lobe lingula lung nodule discovered on a screening radiograph as part of a routine physical examination. The patient denied fever, chills, sweats, anorexia, or weight loss. She had a past medical history of thyroid nodule. Her family history was positive for hypertension, heart disease, and lung cancer. She was taking chlorthalidone for hypertension and ibandronate for osteoporosis as well as multivitamin supplements. Subsequent positron emission tomography showed the hypermetabolic uptake of fluorodeoxyglucose in the nodule with a standard uptake value of 7, which is suspicious for a neoplasm. She underwent wedge resection of this lung nodule. Gross examination showed a 1-cm, grey-tan, firm nodule 0.5 cm from the resection margin. Microscopic examination revealed sheets of oncocytic tumor cells with abundant From the University of South Florida Morsani College of Medicine (WS, RS, MMB) and the Departments of Anatomic Pathology and Sarcoma (MMB), H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida. Submitted March 23, 2015; accepted April 28, 2015. Address correspondence to Marilyn M. Bui, MD, PhD, Moffitt Cancer Center, 12902 Magnolia Drive, Tampa, FL 33612. E-mail: Marilyn.Bui@Moffitt.org No significant relationships exist between the authors and the companies/organizations whose products or services may be referenced in this article. 498 Cancer Control pink, granular cytoplasm, ovoid nuclei, small nucleoli (Fig 1A), and negative surgical margins. Immunohistochemistry was strongly and diffusely positive for DOG1 (Fig 1B) but negative for CD10, CD34, CD117 (C-kit), CD163, cytokeratin (CK) 7, CK20, CK AE1/AE3, thyroid transcription factor 1, epithelial membrane antigen, renal cell carcinoma, thyroglobulin, and ERG. Based on these findings, a preliminary diagnosis of epithelioid gastrointestinal stromal tumor (GIST) was made. Magnetic resonance imaging of the abdomen was obtained and was within normal limits. The patient came to the H. Lee Moffitt Cancer Center & Research Institute (Tampa, FL) for treatment. After performing a thorough history and physical examination and reviewing her imaging and pathology findings, her physician indicated that the findings were inconsistent with a diagnosis of GIST. Criteria for diagnosing GIST are reviewed in Table 1.1 After our pathological review of the case, the DOG1 immunostain (SP31 clone, prediluted via a dispenser) was repeated and it was diffusely but weakly positive (Fig 1C). Is This Gastrointestinal Stromal Tumor? To practice evidence-based medicine, it is necessary to work-up the case in a systemic approach. The steps include: 1. Clinical and radiological correlation with pathology findings: In this case, the overall hisOctober 2015, Vol. 22, No. 4 A Table 1. — Diagnostic Criteria of GIST Site of Involvement Stomach: 54% Small intestine and duodenum: 32% Colon and rectum: 1% Others: 9% Histology Spindle cell tumor, in most Epitheloid: 20%–25% Mixed spindle and epithelial cells Differential Diagnosis Smooth muscle tumor Nerve sheath tumor Other spindle or epitheloid tumor Immunophenotype B CD117 (C-kit) strongly positive, except in 5% of GIST cases with mutant PDGFRA CD34 is positive in most cases of spindle-cell GIST; less consistent in epitheloid histology DOG1 is positive in most cases of GIST (CD117+/DOG1+); often positive in CD117– GIST (CD117– /DOG1+) When to Diagnose GIST? CD117 positivity in context with correct clinical and pathological setting DOG1 more sensitive than CD117 in detecting GIST of gastric origin, epitheloid morphology, and GIST harboring PDGFRA mutation CD34 alone not used to diagnose GIST GIST = gastrointestinal stromal tumor. From Ríos-Moreno MJ, Jaramillo S, Pereira Gallardo S, et al. Gastrointestinal stromal tumors (GISTs): CD117, DOG-1 and PKCθ expression. Is there any advantage in using several markers? Pathol Res Pract. 2012;208(2):74-81. C Fig 1A–C. — (A) Hematoxylin and eosin stain showing an epithelioid neoplasm (× 400). (B) DOG1 (K9 clone) IHC stain showing strong membranous positivity (× 400). (C) DOG1 (SP31 clone) IHC stain showing weak membranous positivity (× 400). IHC = immunohistochemistry. tological features are not typical for epithelioid GIST; however, the immunoreactivity of DOG1 was puzzling.2 Primary GIST of the pleura has been reported in a single case report2; however, primary GIST originating in the lung has not been reported. 2. Review the literature and investigate if DOG1 immunoreactivity is specific for GIST alone and October 2015, Vol. 22, No. 4 what other tumors are positive for DOG1 and could be a potential diagnosis: The literature review revealed an interesting result, and our findings are included below so other pathologists may find them useful when dealing with tumors and GISTs positive for DOG1. 3. Conduct judicious ancillary testing to rule in or out differential diagnoses (eg, carcinoma, melanoma, granular cell tumor, and epithelial sarcoma). 4. If in doubt, seek an expert opinion. After pertinent differential diagnoses were ruled out, we felt this was not a case of epithelioid GIST, but rather a primary lung tumor that warranted further subtyping. Christopher D.M. Fletcher, MD, at Brigham & Women’s Hospital (Boston, MA) was consulted. Other pertinent stains as well as repeat immunostains were negative for DOG1. The tumor was deemed to be an atypical epithelioid neoplasm of the lung; however, this entity is not an established World Health Organization classification but instead is a descriptive diagnosis that reflects the uncertain histogenesis, clinical behavior, Cancer Control 499 and our current scientific understanding of the lesion. The recommended management is judicious follow-up. At the time of publication, the patient is under surveillance. DOG1 has shown rates of high sensitivity and specificity in the detection of GISTs, and 74% of GISTs exhibit a positive DOG1 and CD117 immunoprofile.3 Discovered on GIST-1 (DOG1) protein antibodies are more sensitive than CD117 antibodies in detecting tumors of gastric origin, epithelioid tumors, and tumors harboring PDGFRA.4 Although DOG1 positivity is generally required for the diagnosis of GIST, the results should be interpreted alongside morphological findings of the tumor and the clinical picture, as shown in the current case. From this, we intend to explore the immunoreactivity of DOG1 in non-GIST neoplasms to provide useful information for practicing pathologists and other health care professionals. Methods A search of the English-speaking medical literature was conducted to identify original articles published from 2004 to 2015 regarding the use of the DOG1 antibody for the pathological diagnosis on formalin-fixed and paraffin-embedded human tissue. We reviewed the relevant studies to examine the results of the clones of DOG1 antibodies as well as immunoreactive and tissue types. The clone refers to DOG1 antibodies made by identical immune cells copies of a unique parent cell (K9, SP31, DOG1.1). These clones were developed from mice and rabbits. Documentation of the findings, a critical review of the results, and the application of this information in the work-up of the index case are discussed. Results Antibodies Twelve relevant studies were identified. The DOG1 antibody used in these studies included monoclonal (SP31, K9, DOG1.1), polyclonal, or nonspecified antibodies. Criteria for positivity varied among the studies. DOG1 Expression DOG1 is expressed in normal salivary acini gland and pancreatic endocrine tissue (Table 2).5,6 Table 3 summarizes DOG1 expression in 250 cases out of 2,360 non-GIST neoplasms tested at different anatomical sites.5,7-17 Of the 2,360 tumors tested, the average rate of DOG1 positivity was 10.6%. As illustrated in Fig 2,12,14 DOG1 is 100% positive in cases of renal oncocytoma and chondroblastoma7,9; 60% to 97% positive in acinic cell carcinoma, chromophobe renal cell carcinoma, fibroadenoma, adenoid cystic carcinoma, and squamous cell carci500 Cancer Control noma7,11,12,14; 22% to 53% positive in epithelial-myoepithelial carcinoma, pseudopapillary neoplasm, neoplastic salivary tissue, endometrial adenocarcinoma, gastric adenocarcinoma, and glomus tumor 5,8,10-14; 7% to 17% positive in cholangiocarcinoma, synovial sarcoma, colonic adenocarcinoma, leiomyoma, and pancreatic adenocarcinoma8,10-12,14,15; and 1% to 5% positive in leiomyosarcoma, angiosarcoma, Ewing sarcoma, malignant peripheral nerve sheath tumor, neuroendocrine tumor, melanoma, and schwannoma.5,8,10-12,14-16 Reports regarding solid pseudopapillary neoplasm of the pancreas are conflicting.6,8 Except in cases of renal oncocytoma and chondroblastoma, the tumors mentioned above have various levels of DOG1 expression. However, of the 2,360 cases, a total of 959 tumors, other than those listed in Table 3, are nonresponsive to DOG1 immunohistochemistry. The list of these tumors is summarized in Table 4.7-12,14 Discussion This first clue that this lung tumor may not be GIST is that primary GIST has never been reported.2 If this case was the first discovered primary lung GIST, then molecular confirmation would have been warranted. GIST does not appear to metastasize to the lung, other than in the setting of many years of treatment with tyrosine kinase inhibitors. To the best of our knowledge, primary GISTs in the lung have not been documented in the English literature. The second clue lies in the specificity of DOG1 immunoreactivity in GIST. DOG1 was shown to be independent of the KIT/PDGFRA mutation status and was considered specific for GIST when it was first discovered in 2004.18 In other words, DOG1 should be tested when the tumor is of gastric origin, has epitheloid morphology, and in cases harboring the PDGFRA mutation. Data are emerging regarding the expression of DOG1 in non-GIST tissue. Studies that have reported higher rates of DOG1 specificity Table 2. — DOG1 IHC in Non-Neoplastic Non-GISTs Study Anti-DOG1 Antibody Clone Type Chenevert5 DOG1.1 Ardeleanu6 Polyclonal Site Tumor Type DOG1 Expression, n/N (%) Salivary gland Salivary tissue 109/109 (100) Pancreas Endocrine tissue Adult 11/11 (100) Fetal 15/15 (100) Representative of 135 cases. GIST = gastroinestinal stromal tumor, IHC = immunohistochemistry. October 2015, Vol. 22, No. 4 100 90 80 DOG1 Specificity, % 70 60 50 40 30 20 roblasto Chon d Re n a l o ncocy to ma (n = 0 21) ma (n = Acinic 9) cell c ar C hr o m cinoma ophob e (n = 3 3 r en al c ) ell c arc inoma (n = 37 ) Fibroad enom a (n = 11 Adenoid ) cystic c arcino m a (n S qu a m = 28) ous c e ll c arcin Epitheli o m a (n = 15 al-myo ) epitheli al c ar c in o ma (n= P s eudo 15) papilla r y neop lasm (n En dom = 29) etrioid adeno c arcinom a (n Gastric = 10) adeno c arcinom a (n = 3 9) Glomu s tumo r (n = 2 Neopla 7) stic sa livar y ti ssue (n = 8 3) Cholan gioc arc inoma (n = 6) S ynovia l s ar c o ma (n = Colonic 115) adeno c arcinom a (n = 3 0) Leiomy o ma (n = P a n cr e 10 3) atic ad eno c ar cinoma (n = 11 2) Leiomy os ar c o ma (n = 214) A ngios ar c om a (n = 32 ) Ewing Malign s ar c om a n t p er a (n = 3 ipher al 4) n er ve s heath tu mor (n = 3 4) N e ur o e ndocrin e tumo r (n = 9 9) Melano ma (n= 181) S chw a nnom a (n = 8 3 ) 10 Non-GIST Neoplasm Fig 2. — DOG1 tumor specificity rates. GIST = gastrointestinal stromal tumor. have had small sample sizes, whereas larger studies (> 83 cases) have reported that a small percentage of non-GIST tumors were positive for DOG1 (see Fig 2). 8,10-12,14,16 As shown in Fig 2, neoplastic salivary gland may show DOG1 expression. As shown in Table 3, the average DOG1 specificity rate for neoplastic salivary tissue was 17%.5,11,14 DOG1 expression in synovial sarcoma was reported by 4 different studies to have various specificity rates.10-12,14 The average rates of DOG1 specificity for synovial sarcoma was 14.8%. Gastric adenocarcinoma has 28% positivity for October 2015, Vol. 22, No. 4 DOG1 compared with 13% for colonic adenocarcinoma (see Fig 2).12,14 Even though the location of these tumors may overlap with GIST, they rarely overplay histologically with GIST and do not pose diagnostic challenges. Smooth muscle tumors were reported in 5 studies.10-12,14,15 The average rates of DOG1 specificity for leiomyoma and leiomyosarcoma were 10.7% and 4.2%, respectively (see Fig 2). When the smooth muscle tumor occurs in the gastrointestinal tract, it may be diagnostically challenging to be differentiated from GIST. As shown in Fig 2, nerve sheath Cancer Control 501 Table 3. — DOG1 IHC in Neoplastic Non-GISTs Study Anti-DOG1 Antibody Clone Type Site Tumor Type DOG1 Expression, n/N (%)a Zhao7 Hemminger14 Not specified K9 Kidney Renal oncocytoma Chromophobe renal cell carcinoma 21/21 (100) 32/37 (86) Akpalo9 Wong10 Lopes11 Miettinen12 SP31 K9 Bone Chondroblastoma Glomus tumor Ewing sarcoma 9/9 (100) 6/27 (22) 1/34 (3) Bergman13 Hemminger 8 Not specified K9 Pancreas Pseudopapillary neoplasm Adenocarcinoma Neuroendocrine tumor 15/29 (51) 8/112 (7) 2/99 (2) Chenevert5 Lopes11 Hemminger14 DOG1.1 K9 Head and neck Salivary gland Acinic cell carcinoma Adenoid cystic carcinoma Epithelial-myoepithelial carcinoma Neoplastic salivary tissue 32/33 (97) 20/28 (71) 8/15 (53) 14/83 (17) Lopes11 Wong10 Sah15 Miettinen12 Hemminger14 K9 DOG1.1/K9 Breast Soft tissue Fibroadenoma Synovial sarcoma Leiomyosarcoma Angiosarcoma 9/11 (82) 17/115 (15) 9/214 (4) 1/32 (3) Miettinen12 Sah15 Wong10 Lopes11 K9 Esophagus Stomach Liver Abdomen Squamous cell carcinoma Adenocarcinoma Cholangiocarcinoma Leiomyoma 9/15 (60) 11/39 (28) 1/6 (17) 11/103 (11) Miettinen12 Hemminger14 K9 Colon Adenocarcinoma 4/30 (13) Hemminger14 Wong10 Lopes11 Miettinen12 K9 Uterus (endometrium) Central nervous system Endometrioid adenocarcinoma Malignant peripheral nerve sheath tumor Schwannoma 4/10 (40) 1/34 (3) 1/83 (1) Hemminger14 Gonzalez16 Wong10 Lopes11 Miettinen12 K9/SP31 Skin Melanoma 3/181 (2) Tang17 K9 Prostate Stromal sarcoma 1/1 (100) Representative of 1,401 cases. Combined data from multiple studies. GIST = gastrointestinal stromal tumor, IHC = immunohistochemistry. a tumors also have a small rate of DOG1 positivity (< 5%). In these situations, other markers for GIST, such as CD117, CD34, or mutational analysis for CD117 and PDGFR, can be used to help definitively diagnose GIST. Limitations The various studies we reviewed used different antibodies and various scoring criteria for DOG1 immunoreactivity. Although no specific practice guideline exists for the diagnosis of GIST, strong, diffuse, and membranous DOG1 immunoreactivity was generally accepted for the diagnosis.4 The heterogeneous result 502 Cancer Control of DOG1 immunoreactivity by various laboratories may present a challenge. For example, in our index case, DOG1 was tested in 3 different laboratories with 2 different antibodies and yielded different results. If the DOG1 immunostain was negative, then this lung mass would never have been diagnosed as GIST. Conclusion In addition to DOG1 being detected in cases of gastrointestinal stromal tumor, DOG1 positivity can be detected in other non-neoplastic and neoplastic tissue; however, only in the appropriate clinical and pathological October 2015, Vol. 22, No. 4 Table 4. — IHC Negative for DOG1 in Neoplastic Non-GISTs Study Anti-DOG1 Antibody Clone Type Tumor Type Case, n (N = 959)a Zhao7 Hemminger14 Not specified K9 Clear-cell renal cell carcinoma 35 Akpalo9 Lopes11 SP31 K9/DOG1.1 Chondromyxoid fibroma 12 Giant cell tumor 40 Wong10 Lopes11 Miettinen12 Hemminger14 K9 K9/DOG1.1 K9 K9 Dedifferentiated liposarcoma 27 Desmoid tumor 59 Desmoplastic small round cell tumor 16 Endometrial stromal sarcoma 28 Extraskeletal myxoid chondrosarcoma 16 Gastrointestinal ganglioneuroma 3 Inflammatory fibroid polyp 67 Inflammatory myofibroblastic tumor 23 Kaposi sarcoma 24 Neurofibroma 27 Perivascular epithelioid cell tumor/angiomyolipoma Sarcomatoid carcinoma 8 6 Small cell carcinoma 12 Solitary fibrous tumor 49 Hemminger 8 K9 Serous cystadenoma 28 Lopes K9/DOG1.1 Mesenchymal tumors other than GIST Miettinen12 Lopes11 K9 Extramedullary myeloid tumor 5 Mastocytoma, skin 3 11 Neuroblastoma 10 Seminoma testicular 16 Undifferentiated sarcoma 26 Small intestine carcinoid tumor Undifferentiated sarcomatoid carcinoma Hemminger 8 Lopes11 K9 K9/DOG1.1 261 6 21 Alveolar soft-part sarcoma 3 Chordoma 5 Clear cell sarcoma 5 Epithelioid sarcoma 8 Granular cell tumor 6 Low-grade fibromyxoid sarcoma 8 Lung adenocarcinoma 5 Perineurioma 4 Pleomorphic undifferentiated sarcoma 77 Prostate adenocarcinoma 10 Combined data from multiple studies. GIST = gastrointestinal stromal tumor, IHC = immunohistochemistry. a context is DOG1 positivity specific and helpful for diagnosing true cases of gastrointestinal stromal tumor. References 1. Fletcher CD, Bridge JA, Hogendoorn PC, et al, eds. WHO Classification of Tumours of Soft Tissue and Bone. 5th ed. Lyon, France: InternaOctober 2015, Vol. 22, No. 4 tional Agency for Research on Cancer Press; 2013. 2. Long KB, Butrynski JE, Blank SD, et al. Primary extragastrointestinal stromal tumor of the pleura: report of a unique case with genetic confirmation. A J Surg Pathol. 2010;34(6):907-912. 3. Ríos-Moreno MJ, Jaramillo S, Pereira Gallardo S, et al. Gastrointestinal stromal tumors (GISTs): CD117, DOG-1 and PKCθ expression. Is there any advantage in using several markers? Pathol Res Pract. 2012;208(2):74-81. 4. Lee CH, Liang CW, Espinosa I. The utility of discovered on gastroCancer Control 503 intestinal stromal tumor 1 (DOG1) antibody in surgical pathology-the GIST of it. Adv Anat Pathol. 2010;17(3):222-232. 5. Chenevert J, Duvvuri U, Chiosea S, et al. DOG1: a novel marker of salivary acinar and intercalated duct differentiation. Mod Pathol. 2012;25(7):919-929. 6. Ardeleanu C, Arsene D, Hinescu M, et al. Pancreatic expression of DOG1: a novel gastrointestinal stromal tumor (GIST) biomarker. Appl Immunohistochem Mol Morphol. 2009;17(5):413-418. 7. Zhao W, Tian B, Wu C, et al. DOG1, cyclin D1, CK7, CD117 and vimentin are useful immunohistochemical markers in distinguishing chromophobe renal cell carcinoma from clear cell renal cell carcinoma and renal oncocytoma. Pathol Res Pract. 2015;211(4):303-307. 8. Hemminger J, Marsh WL, Iwenofu OH, et al. DOG1 (clone K9) is seldom expressed and not useful in the evaluation of pancreatic neoplasms. Appl Immunohistochem Mol Morphol. 2012;20(4):397-401. 9. Akpalo H, Lange C, Zustin J. Discovered on gastrointestinal stromal tumour 1 (DOG1): a useful immunohistochemical marker for diagnosing chondroblastoma. Histopathology. 2012;60(7):1099-1106. 10. Wong NA, Shelley-Fraser G. Specificity of DOG1 (K9 clone) and protein kinase C theta (clone 27) as immunohistochemical markers of gastrointestinal stromal tumour. Histopathology. 2010;57(2):250-258. 11. Lopes LF, West RB, Bacchi LM, et al. DOG1 for the diagnosis of gastrointestinal stromal tumor (GIST): comparison between 2 different antibodies. Appl Immunohistochem Mol Morphol. 2010;18(4):333-337. 12. Miettinen M, Wang ZF, Lasota J. DOG1 antibody in the differential diagnosis of gastrointestinal stromal tumors: a study of 1840 cases. Am J Surg Pathol. 2009;33(9):1401-1408. 13. Bergmann F, Andrulis M, Hartwig W, et al. Discovered on gastrointestinal stromal tumor 1 (DOG1) is expressed in pancreatic centroacinar cells and in solid-pseudopapillary neoplasms--novel evidence for a histogenetic relationship. Hum Pathol. 2011;42(6):817-823. 14. Hemminger J, Iwenofu OH. Discovered on gastrointestinal stromal tumours 1 (DOG1) expression in non-gastrointestinal stromal tumour (GIST) neoplasms. Histopathology. 2012;61(2):170-177. 15. Sah SP, McCluggage WG. DOG1 immunoreactivity in uterine leiomyosarcomas. J Clin Pathol. 2013;66(1):40-43. 16. Gonzalez RS, Carlson G, Page AJ, et al. Gastrointestinal stromal tumor markers in cutaneous melanomas: relationship to prognostic factors and outcome. Am J Clin Pathol. 2011;136(1):74-80. 17. Tang YL, Wong CF, Yap WM, et al. Discovered on gastrointestinal stromal tumours 1 (DOG1) expression in non-gastrointestinal stromal tumour (non-GIST) neoplasms. Histopathology. 2014;65(5):724-726. 18. West RB, Corless CL, Chen X, et al. The novel marker, DOG1, is expressed ubiquitously in gastrointestinal stromal tumors irrespective of KIT or PDGFRA mutation status. Am J Pathol. 2004;165(1):107-113. 504 Cancer Control October 2015, Vol. 22, No. 4 Case Report Occurrence of Multiple Tumors in a Patient Elaine Tan, Mark Friedman, MD, and Domenico Coppola, MD Summary: An 81-year-old man initially presented with a right forearm mass that was found to be myxofibrosarcoma. In addition, he was found to have gastric and intragastric masses identified as neuroendocrine tumor (NET) and gastrointestinal stromal tumor (GIST; presenting synchronously), respectively, as well as a new left upper quadrant mass identified as desmoid tumor in the colon. The patient complained of melena, which was found to be due to metastatic myxofibrosarcoma in the transverse colon. Several reports have associated GIST with NET and some reports have associated GIST with sarcomas and NET with sarcomas; however, this is the first report to document all these tumors in a single patient. Several factors may have contributed to the development of these tumors, including growth factors secreted by NET, KIT mutation of GIST predisposing to additional tumors, immunosuppressed state, or an underlying genetic syndrome. This case highlights the importance of investigating for additional malignancies when a primary malignancy is discovered. Case Report An 81-year-old man with a past medical history significant for multifocal lipomas and prostate cancer, for which he underwent prostatectomy, presented for removal of a mass on his right forearm. Excision was performed and pathology revealed high-grade myxofibrosarcoma. The resected tumor was 5 cm in size. Histologically, the tumor was poorly differentiated and involved the surgical resection margins. The tumor was composed of large epithelioid cells and spindle cells with scattered multinucleated cells. Nuclear pleomorphism was present, and the surrounding stroma was myxoid and desmoplastic. The patient underwent wide resection and radiotherapy. Positron emission tomography/computed tomography demonstrated a large, hypermetabolic, soft-tisFrom the Departments of Anatomic Pathology (ET, DC) and Gastroenterology (MF), H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida. Submitted July 20, 2015; accepted July 29, 2015. Address correspondence to Domenico Coppola, MD, Department of Anatomic Pathology, Moffitt Cancer Center, 12902 Magnolia Drive, Tampa, FL 33612. E-mail: Domenico.Coppola@Moffitt.org No significant relationships exist between the other authors and the companies/organizations whose products or services may be referenced in this article. October 2015, Vol. 22, No. 4 sue mass in the subcutaneous fat of the posterior thigh and a hypermetabolic mass in the left upper quadrant of the abdomen. Wide excision of the mass of the posterior thigh revealed a 6-cm, metastatic, high-grade myxofibrosarcoma with positive margins. The patient underwent radiotherapy. Later the same year, the patient underwent esophagogastroduodenoscopy, which revealed a gastric mass seen at the greater curvature; partial gastrectomy was performed. Macroscopically, a 5.5 × 3.2 × 3.2 cm well-circumscribed, soft, tan-colored, lobulated gastric mass was identified. The tumor was composed of a monomorphic population of cells with granular pink cytoplasm and round vesicular nuclei with nucleoli (Fig 1A). Immunohistochemically, the tumor was diffusely positive for cytokeratin (Fig 1B), chromogranin, and synaptophysin (Fig 1C), and Ki-67 revealed 4% positivity in 4% of the tumor (Fig 1D). These findings supported a diagnosis of well-differentiated, grade 2 neuroendocrine tumor (NET). One of 4 regional lymph nodes was positive for metastatic NET. In addition, a 2.5 × 1.3 × 1.1 cm, well-circumscribed, firm, tan-colored intragastric mass composed of spindle cells, arranged in intersecting fascicles and focally exhibiting paranuclear vacuoles (Fig 2A), was seen. These cells were diffusely positive for CD117 (Fig 2B) and CD34 (Fig 2C) but negative for actin (Fig 2D) and S100 by immunohistochemistry. The Ki-67 proliferation index was 1%. Genetic analysis of the mass revealed a mutation in exon 11 of KIT. Based on these findings, the diagnosis of gastrointestinal stromal tumor (GIST) was made. More than 2 years after the patient originally presented, computed tomography of his abdomen and pelvis showed a new mass in the left upper quadrant of his abdomen, so segmental colectomy with primary colocolostomy was performed. The tumor was 3 cm in its largest diameter and had negative margins. Gross examination revealed a white, dense mass invading the muscularis propria of the large bowel. Microscopically, the tumor was composed of plump, fibroblastic spindle cells infiltrating the smooth muscle of the muscularis propria (Fig 3A). No necrosis or mitotic activity was present. The lesion was completely resected. The lesional cells were immunohistochemically positive for beta-catenin (Fig 3B) and negative for S100, CD117, acCancer Control 505 A C similar to this patient’s primary high-grade myxofibrosarcoma located in his right forearm and had infiltrated the colonic wall, including the colonic mucosa (Fig 4). Discussion GISTs have been well documented to be associated with the development of synchronous primary tumors, and this rate of associaB D tion can be as high as 11.5% to 33.3%.1 Oftentimes, GIST coexists with adenocarcinoma of gastric, colonic, or pancreatic origin, as well as breast, prostate, and lung cancers.2,3 In our patient, GIST was found in association with gastric NET, a finding that has been reported a handful of times. In Fig 1A–D. — Gastric neuroendocrine tumor, ×100 magnification. (A) Monotonous population of cells 1 case report, a 69-year-old man with small round nucleoli exhibiting a “salt and pepper” chromatin pattern. Hematoxylin and eosin stain. with well-differentiated NET in (B) Low-molecular-weight cytokeratin IHC. (C) Synaptophysin IHC. (D) Ki-67 proliferative index (≤ 4%) IHC. the corpus was also found to harIHC = immunohistochemistry. bor submucosal borderline GIST.4 In another case, a 65-year-old A C woman with GIST of the gastric corpus was diagnosed with concomitant, well-differentiated NET in the same location.5 Although these 2 cases are examples of GIST being synchronous to NET, other cases have described a single tumor type that predated the occurrence of the second tumor by years; however, no clear preB D disposition of one tumor leading to the other has been established.2,6 GISTs have also been found in association with sarcomas. Arnogiannaki et al1 reported the simultaneous occurrence of gastric GIST with uterine leiomyosarcoma. Similarly, Sharma et al7 reported a case of a 50-year-old Fig 2A–D. — Intragastric gastrointestinal stromal tumor, ×200 magnification (A) Intersecting fascicles woman with ileal GIST and synof spindle cells with elongated nuclei with blunt ends. Hematoxylin and eosin stain. (B) CD117 IHC. chronous, high-grade breast (C) CD34 IHC. (D) Actin IHC. IHC = immunohistochemistry. sarcoma with heterologous cartilaginous, osseous, and rhabdotin, and desmin. These results supported the diagnosis myoblastic differentiation. An association with NET of a desmoid tumor (fibromatosis). and sarcoma has also been described by Adams et al8 Nine months later, the patient represented with who reported the occurrence of embryonal rhabdomelena, and subsequent colonoscopy revealed a polmyosarcoma of the cervix and appendiceal carcinoid ypoid lesion in the transverse colon measuring 3.5 cm tumor in a 43-year-old woman. However, our patient in its largest diameter. Histologically, the tumor was had a unique combination of myxofibrosarcoma, GIST, 506 Cancer Control October 2015, Vol. 22, No. 4 A B Fig 3A–B. — Desmoid tumor. (A) Proliferation of myofibroblasts exhibiting vesicular nuclei and separated by large amount of stroma. Hematoxylin and eosin stain, ×200 magnification. (B) Beta catenin, ×400 magnification. addition, a primary malignancy may create an immunosuppressed state, thus causing patients to be more susceptible to developing secondary tumors.10 The presence of genetic mutations could also lead to the simultaneous occurrence of these tumors, similar to how cancer syndromes like Li–Fraumeni syndrome, von Hippel–Lindau syndrome, and multiple endocrine neoplasia syndromes result from genetic mutations. Exposure to carcinogenic agents might also lead to the development of these synchronous tumors or, possibly, the synchrony happened by pure coincidence.3 Conclusion Gastrointestinal stromal tumors (GISTs) tend to present with synchronous tumors. When a patient is diagnosed with a non-GIST malignancy, the health care professional must consider the possibility of a synchronous or metachronous GIST, among other types of tumors. More research is needed on the pathophysiology of these synchronous and metachronous tumors. Fig 4. — Metastatic high-grade myxofibrosarcoma. Multinucleated spindle-shaped cells surrounded by myxoid and desmoplastic stroma infiltrating the wall and mucosa of the colon. Hematoxylin and eosin stain, ×200 magnification. NET, and desmoid tumor. To our knowledge, no other case of this kind has been previously reported in the English literature. Several hypotheses have been postulated to explain the development of synchronous and metachronous primary tumors in the same patient. Two studies were unable to establish an immunohistochemical or molecular profile difference between single GISTs and GISTs coexisting with other tumors.1,3 The secretion of growth factors from NETs could contribute to the development of concurrent primary tumors. Growth factors such as platelet-derived growth factor, transforming growth factor β, and basic fibroblast growth factor are secreted from NETs and are all tumorigenic.9 GISTs are characterized by the KIT mutation that may predispose them to the development of other tumors; moreover, impaired KIT expression has been noted in carcinomas of the breast and colon.1 In October 2015, Vol. 22, No. 4 References 1. Arnogiannaki N, Martzoukou I, Kountourakis P, et al. Synchronous presentation of GISTs and other primary neoplasms: a single center experience. In Vivo. 2010;24(1):109-115. 2. Samaras VD, Foukas PG, Triantafyllou K, et al. Synchronous well differentiated neuroendocrine tumour and gastrointestinal stromal tumour of the stomach: a case report. BMC Gastroenterol. 2011;11:27. 3. Liszka L, Zielinska-Pajak E, Pajak J, et al. Coexistence of gastrointestinal stromal tumors with other neoplasms. J Gastroenterol. 2007;42(8):641-649. 4. Maiorana A, Fante R, Maria Cesinaro A, et al. Synchronous occurrence of epithelial and stromal tumors in the stomach: a report of 6 cases. Arch Pathol Lab Med. 2000;124(5):682-686. 5. Lin YL, Wei CK, Chiang JK, et al. Concomitant gastric carcinoid and gastrointestinal stromal tumors: a case report. World J Gastroenterol. 2008;14(39):6100-6103. 6. Hung CY, Chen MJ, Shih SC, et al. Gastric carcinoid tumor in a patient with a past history of gastrointestinal stromal tumor of the stomach. World J Gastroenterol. 2008;14(44):6884-6887. 7. Sharma M BK, Barad AK, Padu K, et al. Spontaneous Perforation as a First Presentation of Ileal Gastrointestinal Stromal Tumour (GIST) with Synchronous Breast Sarcoma. J Clin Diagn Res. 2014; 8(5): ND07- ND09. 8. Adams BN, Brandt JS, Loukeris K, et al. Embryonal rhabdomyosarcoma of the cervix and appendiceal carcinoid tumor. Obstet Gynecol. 2011;117:482-484. 9. Chaudhry A, Funa K, Oberg K. Expression of growth factor peptides and their receptors in neuroendocrine tumors of the digestive system. Acta Oncol. 1993;32(2):107-114. 10. Habal N, Sims C, Bilchik AJ. Gastrointestinal carcinoid tumors and second primary malignancies. J Surg Oncol. 2000;75(4):310-316. Cancer Control 507 Special Report Association of Cancer Stem Cell Markers With Aggressive Tumor Features in Papillary Thyroid Carcinoma Zhenzhen Lin, MD, Xuemian Lu, MD, Weihua Li, MD, Mengli Sun, MD, Mengmeng Peng, MD, Hong Yang, MD, Liangmiao Chen, MD, Chi Zhang, PhD, Lu Cai, MD, PhD, and Yan Li, MD, PhD Background: Identifying accurate prognostic molecular markers for papillary thyroid carcinoma (PTC) is important because many patients with PTC may be erroneously considered to have low-risk tumors. Evidence is also accumulating to support the existence of cancer stem cells in PTC. Methods: Thirty controls and 167 patients with PTC were selected to establish a tissue microarray to investigate cancer stem cell marker expression in samples from an established pathological database. The protein expressions of CD44, CD133, epithelial cell adhesion molecule (EpCAM), CD45, and CD90 were evaluated by immunohistochemical assay in the tissue microarray. Results: The protein levels of CD44, CD133, and EpCAM were significantly increased in PTC tissue compared with tissue from the controls. A positive correlation was found between cancer stem cell markers and tumor, node, and metastasis staging. Conclusions: Among a subset of patients with PTC, cancer stem cells detected by immunohistochemistry can be used as prognostic markers to screen for potential tumor dissemination. Whether these cancer stem cell markers are potentially therapeutic targets — and, thus, could be used for effective adjuvant treatment strategies — remains to be seen, and more data are needed. Introduction Papillary thyroid carcinoma (PTC) is the most common endocrine malignancy, and an estimated 62,450 new cases of thyroid cancer will be diagnosed in 2015 in the United States alone; of these diagnoses, 3 out of 4 cases will occur in women.1,2 In general, PTC has a favorable prognosis, the overall 10-year survival rate of patients with PTC is about 90%, however, approximately 10% and 20% of patients with stage 1 or 2 PTC, respectively, have disease recurrence. 2 Tumor recurrence affects the quality of life of patients; among those who develop local recurrence, some will die from the disease, indicating the existence of more aggressive variants of PTC. Up to 30% of patients with differentiated thyroid carcinoma may have tumor recurrence within several decades; 66% From the Department of Endocrinology (ZL, XL, WL, MS, MP, HY, LC, CZ), Third Affiliated Hospital, Wenzhou Medical University, Ruian, Zhejiang, China, Department of Surgery (YL), School of Medicine, University of Louisville, Louisville, Kentucky, ChineseAmerican Research Institute for Diabetic Complications (XL, CZ, LC), Ruian Center, Ruian, Zhejiang, China, and Kosair Children’s Hospital Research Institute (LC), Department of Pediatrics, University of Louisville, Louisville, Kentucky. Submitted March 13, 2015; accepted July 24, 2015. Address correspondence to Xuemian Lu, MD, Department of Endocrinology, The Third Affiliated Hospital of Wenzhou Medical University, Ruian, Zhejiang, China, 325200. E-mail: lu89118@ medmail.com.cn This project was partly supported by the Science and Technology Program of Ruian, China (No. 201203074). No significant relationships exist between the authors and the companies/organizations whose products or services may be referenced in this article. 508 Cancer Control of recurrences occur within the first decade following initial therapy.3 Therefore, identifying the subpopulation at high-risk for disease recurrence is necessary. Based on clinical and pathological studies, various factors, such as age, sex, tumor size, extrathyroidal extension, and distant metastasis, have been investigated to determine whether an association exists between them and PTC recurrence; however, the matter is controversial.4 Molecular and cellular events have been shown to be associated with PTC, and the cancer progenitor/stem cell is considered by some as a common cellular alteration detected in patients with PTC.5,6 Cancer stem cells, a small subpopulation of cells with stemlike properties, are key factors for tumor initiation and recurrence.7 Evidence supporting the existence of cancer stem cells in PTC is rapidly accumulating.8-12 Recent research indicates that increased cancer stem cell–like features play an important role in the progression of PTC.13 The cancer stem cell theory was established based on the observation that cancer cell populations are heterogeneous.14 The identification and characterization of cancer stem cells in tumors largely depend on the surface markers shared with normal stem cells.15 These populations have been identified and isolated using the surface markers CD44 and CD24 in a human PTC cell line, TPC-1 (BHP10-3).2 Research shows that POU5F1, a stem cell marker, is significantly higher in CD44+ (high levels of CD44) and CD24 Lin (low levels of CD24) cells, supporting the hypothesis that CD44+CD24–Lin– cells are potential cancer October 2015, Vol. 22, No. 4 stem cells.2 Further study suggests that CD44+CD24 – Lin– PTC cells can form thyrospheroids, which are important in vitro features of cancer stem cells; moreover, data indicate that thyrospheroids can initiate a tumor in immunodeficient mice.2 Several other potential stem cell markers have been used by others in thyroid cancer stem cell research, such as CD133 and epithelial cell adhesion molecule (EpCAM).5,16-18 Although the prognostic influence of cancer stem cells in PTC is unclear, the use of cancer stem cell markers may help histopathologically determine the existence of cancer stem cells in PTC tissue, possibly impacting patient prognosis. Traditional immunohistological examination consists of single-sectioning paraffin blocks and is amenable to routine pathology; however, a wide variety of the staining condition by traditional assay may limit its utility for detecting cancer stem cell markers in resected PTC samples. Tissue microarray is a high-throughput technology used for analyzing molecular markers in oncology. It offers added benefit with respect to decreased technical variability during the staining and interpretation process. In our study, we established a tissue microarray process to investigate the expressions of cancer stem cell markers in tissue samples from a pathological database of patients with PTC. The specimens used in the tissue microarray consisted of different pathological stages of PTC and were stained by immunohistochemical assay. The protein expressions of CD44, CD133, EpCAM, CD45, and CD90 were then evaluated for each pathological stage of PTC. Furthermore, we set forth to determine the correlation between cancer stem cell markers and the pathological stages of PTC. Materials and Methods Thyroid nodule tissue from 670 patients was collected from a pathological database maintained by the Third Affiliated Hospital of Wenzhou Medical University (Ruian, Zhejiang, China). All specimens with a diagnosis of PTC were collected following surgical excision. In this retrospective study, we reviewed the database for patients undergoing thyroid nodule resection between August 2012 and September 2013. This study was approved by the Institutional Review Board at Wenzhou Medical University. Patient Selection Patients were excluded if they had radiotherapy, chemotherapy, or both, either preoperatively or postoperatively. Patients also met exclusion criteria if they had certain types of conditions, including autoimmune disease, cerebrovascular accident, coronary artery disease, viral hepatitis, or another type of cancer. Overall, 167 patients postoperatively diagnosed with PTC, along with 30 benign, non-PTC controls, met inclusion October 2015, Vol. 22, No. 4 criteria based on the completeness of their data. Dates of diagnoses and collection times ranged from August 2012 to September 2013. Patients were divided into 4 treatment groups according to tumor, node, and metastasis (TNM) staging per the classification of thyroid cancer by the American Joint Committee on Cancer.19 PTC stage 1 was defined as papillary carcinoma localized to the thyroid gland. PTC stage 2 was defined as either (a) papillary carcinoma that has spread distantly in patients younger than 45 years, or (b) papillary carcinoma larger than 2 cm but no larger than 4 cm, and was limited to the thyroid gland in patients older than 45 years. PTC stage 3 was defined as papillary carcinoma occurring in patients older than 45 years that was larger than 4 cm and was limited to the thyroid or with minimal extrathyroidal extension, or positive lymph nodes limited to the pretracheal, paratracheal, or prelaryngeal/Delphian nodes. PTC stage 4 was defined as papillary carcinoma in patients older than 45 years that extended beyond the thyroid capsule to the soft tissues of the neck, cervical lymph node metastases, or distant metastases. Of the 167 patients, 139 (83.2%) were women and 28 (16.8%) were men. Of the non-PTC controls with resected thyroid nodules, 26 (86.6%) were women and 4 (13.3%) were men. The mean age of the 167 study patients was 50.21 ± 11.65 years compared with 48.9 ± 11.34 years for the controls. Of the patients with PTC, 11 had a family history of diabetes mellitus, 26 had a family history of hypertension, 24 had a history of Hashimoto thyroiditis (chronic lymphocytic thyroiditis), and 3 were former smokers; by contrast, no controls had family histories of diabetes mellitus, hypertension, and smoking, and 1 had a history of Hashimoto thyroiditis history. Medical records were also reviewed (Table 1). Tissue Microarrays The surgical resection of PTC samples provided ample tissue for paraffin-embedded tissue blocks, and tissue array was constructed from the paraffin blocks. Prior to constructing the tissue arrays, the blocks were categorized into 5 groups: non-PTC (“benign controls”) and stages 1 to 4. The categorization was made according to each study patient’s clinical history and diagnosis. To avoid sample-selection bias, 2 pathologists reviewed the hematoxylin and eosin–stained tissue sections to identify areas of PTC and select the regions in blocks as donor core tissues. The reviewers were blinded to the study patients’ clinical history. Donor core tissues with a diameter of 1 mm were obtained from corresponding regions in the donor paraffin block and embedded into a recipient block. Cores from archived specimens of normal thyroid tissue adjacent to Cancer Control 509 Table 1. — Demographics of Study Patient and Control Groups (N = 197) Characteristic Men:women Papillary Thyroid Carcinoma (n = 167) Control (n = 30) 28:139 4:26 50.21 ± 11.65 48.9 ± 11.34 Family history of diabetes mellitus, number ratio 11 (167) 0 (30) Family history of hypertension, number ratio 26 (167) 0 (30) History of smoking, number ratio 3 (167) 0 (30) History of Hashimoto thyroiditis, number ratio 24 (167) 1 (30) Thyroid-stimulating hormone, mU/L 2.02 ± 6.87 1.26 ± 0.85 Total thyroxine 3, nmol/L 1.16 ± 0.26 1.12 ± 0.28 Total thyroxine 4, nmol/L 78.86 ± 36.92 88.61 ± 21.83 Free thyroxine 3, pmol/L 3.09 ± 0.62 2.93 ± 0.45 Age, y Free thyroxine 4, pmol/L 8.66 ± 4.84 9.13 ± 3.05 Glucose, mmol/L 5.65 ± 1.86 5.39 ± 0.63 Triglycerides, mmol/L 1.95 ± 1.44 1.59 ± 0.71 Total cholesterol, mmol/L 5.09 ± 0.99 5.25 ± 0.97 the thyroid nodule were provided as non-PTC controls. Single-donor core tissue samples from each specimen consisting of non-PTC (“benign controls”) and stages 1 to 4 were sampled and designed as 4 rows × 6 columns per block. The donor cores from all study patients were embedded in a tissue microarray recipient block. Nine tissue microarray recipient blocks were prepared consisting of tissue cores obtained from PTC and nonPTC samples. Immunohistochemical Assay Immunohistochemical staining was obtained on the paraffin-embedded blocks of tissue arrays using the DAKO EnVision+ System Kit (DAKO, Carpinteria, California). In brief, the sections were deparaffinized and hydrated. The slides were washed with a Tris buffer, and peroxidase blocking was performed for 5 minutes. After rewashing, the monoclonal antibodies, CD44 and EpCAM, and polyclonal antibodies, CD45, CD90, and CD133, were applied for 60 minutes at room temperature. The slides were rinsed and incubated with labeled polymer for 30 minutes at room temperature. The substrate-chromogen solution (diaminobenzidine) was added as a visualization reagent. As a final step, the slides were counterstained with hematoxylin. A negative control, using the same experimental procedure as the tested slides, except for the primary antibody, was included in each run. 510 Cancer Control Computer Image Analysis Computer image analysis was performed to quantify the expressions of the cancer stem cell markers in 167 study patients with PTC and 30 benign controls. Two pathologists performed a pathological reading to confirm the PTC diagnosis for each tissue microarray slide before the digital image was captured. The imaging fields were randomly chosen from each core tissue to ensure sampling objectivity. Five imaging fields were scanned for each specimen sample. All digital images were acquired with the Olympus Microscope IX51 (Olympus, Pittsburgh, Pennsylvania) at 200 magnification using the Spot camera via the Image-Pro Plus Imaging System (Media Cybernetics, Warrendale, Pennsylvania) and stored as jpg data files (resolution was fixed at 200 pixels per inch). The acquired color images from immunohistochemical staining were defined as standard threshold according to software specifications. The software quantified the threshold area represented in these images. Either the PTC images or normal thyroid images were delineated from the obtained tissues as a region of interest (ROI) using the set color threshold subroutine of the image analysis software. Because of the existence of the duct structure of PTC — in particular, the benign thyroid tissue — the “no tissue” regions were excluded within the ROI to avoid faulty measurement. The integrated optical density measured for positive staining was a representation of the mass and a measurement of the total amount of positive staining in the delineated regions. Values of integrated optical density were normalized by dividing by the area of the threshold ROI of the positive staining. Positive expression was represented as the digital values of the integrated optical density/area, allowing us to analyze the statistical significance among the study groups. Statistic Analysis Analysis of variance was performed using SPSS16.0 (IBM, New York, New York) to determine the statistical significance of the level of expression of the cancer stem cell markers, if any, between TNM staging and controls. Spearman rank correlation coefficient was performed to analyze the correlation between cancer stem cell markers and TNM staging. Values are reported as mean plus or minus standard deviation. Differences between groups were regarded as statistically significant when P values were less than .05. Results Protein Expressions of CD44, CD133, EpCAM, CD45, and CD90 The expression pattern of CD44 showed that the positive staining mainly presented on the cell October 2015, Vol. 22, No. 4 membrane of PTC cells. Strong staining of CD44 was found in all PTC tissue, but weak and negative staining were both found in the tissue from the benign controls. Most PTC cells in stages 3 and 4 with strong CD44 staining also showed cytomorphological features of poor polarity, which was characterized by the loss of both apical-basal polarity and wellarranged order of neighboring cells. By contrast, PTC cells in stages 1 and 2 had cytomorphologic features of well polarity with weaker staining of CD44. The computer quantification of CD44 expression in control specimens was 56.4 ± 29.88. Compared with the control specimens, significant increases of CD44 expression were seen in specimens with stage 1 (241.71 ± 87.25; P < .01), stage 2 (311.98 ± 135.26; P < .01), stage 3 (354.71 ± 103.42; P < .01), and stage 4 (374.58 ± 51.6; P < .01; Fig 1). The expression pattern of CD133 showed that the positive staining mainly presented in the cytosol of PTC cells. Similar to CD44 expression, strong staining of CD133 was found in all PTC tissue, but weak and negative staining was found in the tissue from the benign controls. The PTC cells from stages 3 and 4 tissue samples had poor polarity with strong CD133 staining, whereas the PTC cells from stages 1 and 2 tissue samples had well polarity with weaker staining of CD133. The computer quantification of CD133 expression in the control specimens was 52.64 ± 37.45. Compared with the controls, CD133 expression slight- ly increased in the PTC tissue samples with stage 1 (152.2 ± 52.72; P > .05) but significantly increased in the PTC tissue samples with stage 2 (201.18 ± 60.33; P < .01), stage 3 (242.49 ± 66.81; P < .01), and stage 4 (284.81 ± 87.63; P < .01; Fig 2). EpCAM showed a similar expression pattern as CD133, in which the positive staining mainly presented in the cytosol of PTC cells. Strong staining of EpCAM was seen in all PTC malignant tissue samples but weak and negative staining was seen in the benign controls. The computer quantification of EpCAM expression in control specimens was 48.30 ± 27.07. Compared with the controls, EpCAM expression increased in the stage 1 (157.48 ± 56.48; P > .05) PTC tissue samples but significantly increased in the PTC tissue samples with stage 2 (191.33 ± 51.12; P < .01), stage 3 (201.83 ± 58.20; P < .01), and stage 4 (226.75 ± 81.44; P < .01; Fig 3). The expressions of CD45 and CD90 showed weak (CD45) and mild staining (CD90) in the PTC tissue samples from all 4 stages as well as the tissue samples from the benign controls. Analysis of variance did not indicate a statistical significance among the TNM stages and controls (Table 2). Correlation of CD44, CD133, and EpCAM We further analyzed the correlation between the 3 cancer stem cell markers (CD44, CD133, and EpCAM) and TNM staging. Positive correlations were B D E C CD44 Expression (IOD/area) A 800 F 600 400 200 0 Stage Stage Stage Stage Benign 1 2 3 4 Controls Fig 1A–F. — Detection of CD44 expressions by immunohistochemistry and computer image analysis of positive staining in the papillary thyroid carcinoma tissue of TNM stages as well as in the tissue of benign controls. (A) Stage 1 tumor. (B) Stage 2 tumor. (C) Stage 3 tumor. (D) Stage 4 tumor. (E). Benign controls. (F). CD44 expression. *P < .05 vs benign controls. IOD = integrated optical density, TNM = tumor, lymph mode, metastasis. October 2015, Vol. 22, No. 4 Cancer Control 511 B D E C CD133 Expression (IOD/area) A F 500 400 , , 300 200 100 0 Stage Stage Stage Stage Benign 1 2 3 4 Controls Fig 2A–F. — Detection of CD133 expression by immunohistochemistry and computer image analysis of positive staining in the papillary thyroid carcinoma tissue of 4 TNM stages and benign controls. (A) Stage 1 tumor. (B) Stage 2 tumor. (C) Stage 3 tumor. (D) Stage 4 tumor. (E). Benign controls. (F). CD44 expression. *P < .05 vs benign controls. **P < .05 vs stage 1. IOD = integrated optical density, TNM = tumor, lymph mode, metastasis. B D E C EpCAM Expression (IOD/area) A 500 F 400 300 200 100 0 Stage Stage Stage Stage Benign 1 2 3 4 Controls Fig 3A–F. — Detection of EpCAM expressions by immunohistochemistry and computer image analysis of positive staining in the papillary thyroid carcinoma tissue of TNM stages as well as in the tissue of benign controls. (A) Stage 1 tumor. (B) Stage 2 tumor. (C) Stage 3 tumor. (D) Stage 4 tumor. (E). Benign controls. (F). CD44 expression. *P < .05 vs benign controls. EpCAM = epithelial cell adhesion molecule, IOD = integrated optical density, TNM = tumor, lymph mode, metastasis. 512 Cancer Control October 2015, Vol. 22, No. 4 Variable No. of Patients Intensity of IOD (mean ± SD) P value CD45 CD90 Age, y .375 < 45 57 54.43 ± 60.28 1.63 ± 2.07 ≥ 45 110 65.14 ± 79.93 1.74 ± 2.34 Sex .937 Male 28 60.47 ± 66.1 2.38 ± 2.75 Female 139 61.69 ± 75.48 1.57 ± 2.11 TNM stage CD45: .745 CD90: .309 1 101 59.95 ± 71.31 1.76 ± 2.26 2 14 81.30 ± 103.95 1.00 ± 1.24 3 48 60.23 ± 72.06 1.66 ± 2.22 4 4 45.75 ± 37.97 3.36 ± 4.36 30 8.14 ± 7.7 1.41 ± 1.73 Controls IOD = integrated optical density, SD = standard deviation, TNM = tumor, lymph mode, metastasis. found between CD44 and TNM staging (r 2 = 0.5138; P < .01; Fig 4A), between CD133 and TNM staging (r 2 = 0.6107; P < .01; Fig 4B), and between EpCAM and TNM staging (r 2 = 0. 27; P < .01; Fig 4C). Discussion The results from this study demonstrate significant increases of expressions of CD44, CD133, and EpCAM in PTC tissue samples compared with the tissue samples from the benign controls. The increased amounts of these cancer stem cell surface markers were moderate in stage 1 but were the most pronounced in stages 2 to 4 of PTC. To our knowledge, this is the first study to examine the relationship between cancer stem cell surface markers and TNM staging of PTC. To date, CD44, CD133, and EpCAM, which were initially described as surface markers for malignant 600 400 200 0 Benign Stage Stage Stage Stage Controls 1 2 3 4 500 A CD133 Expression CD44 Expression 800 progenitor cells, have been widely explored as reliable markers for isolating cancer stem cells in many tumor types.20 Interest in these cancer stem cell markers has increased with the observation that the overexpression of cancer stem cell surface markers in tumor cells is associated with therapeutic failure and tumor recurrence.21 In PTC, immunohistochemical staining of CD44 has been shown to have a strong membranous expression pattern, which is consistent with our results.22 In addition, other studies show that the protein levels of CD44 significantly increase in biopsy tissue samples of most patients with PTC.22-24 Accordingly, in our study, the overall expression of CD44 was increased among all patients with PTC. Research has shown that a CD133-positive subpopulation, such as cancer-initiating cells, can reproduce an original tumor and serially transplant it for several generations in immunodeficient mice, whereas CD133-negative cells cannot form tumors.25 Despite CD133 being known as a cancer stem cell marker in many tumors and tissue, CD133 expression in PTC was detected at a lower level in PTC cell lines and in patients with PTC; however, it was detected at a higher level in patients with anaplastic thyroid carcinoma, which is a highly aggressive neoplasm.26-28 With regard to subcellular compartmentalization, we found CD133 expression to be predominantly cytoplasmic, which is consistent with previous observations.28 In our observations, very weak CD133 expression was detected in normal human thyroid tissue, and a lower level of CD133 expression was detected in patients with stages 1 and 2 PTC. However, a high level of CD133 expression was detected in patients with stages 3 and 4 stage PTC. Cytomorphological features of stages 3 and 4 PTC showed poor polarity, which is consist with its aggressive growing pattern, in which tumor growth was found beyond the thyroid capsule, including metastasis to the soft tissues of the neck, cervical lymph node metastases, and distant metastases. Although lymphatic spread is the most common form of PTC progression, the existing residual micrometastatic tumor cells in fact contribute to the 400 300 200 100 0 Benign Stage Stage Stage Stage Controls 1 2 3 4 400 B EpCAM Expression Table 2. — Expressions of CD45 and CD90 in Study Patients With Papillary Thyroid Carcinoma C 300 200 100 0 Benign Stage Stage Stage Stage Controls 1 2 3 4 Fig 4A–C. — Positive correlation of (A) CD44, (B) CD133, and (C) EpCAM expression in TNM staging. EpCAM = epithelial cell adhesion molecule, TNM = tumor, lymph mode, metastasis. October 2015, Vol. 22, No. 4 Cancer Control 513 relapse.29 Residual, micrometastatic tumor cells are undetectable by conventional histopathological examination. Previously, immunohistochemistry using the monoclonal anti-EpCAM antibody (Ber EP4) for visualizing disseminated tumor cells in “tumor free” lymph nodes was performed by others.18 In that study, positive EpCAM tumor cells were revealed among “tumor-free” lymph nodes in 12.5% of cases.18 EpCAM is a 40kDa transmembrane glycoprotein frequently overexpressed in cancer progenitor cells, which are cancer-initiating cells in many human malignancies, including PTC and — although at very lower levels — in normal epithelia.18,30,31 Thus, the finding of positive EpCAM expression in tumor cells among “tumor free” lymph nodes may be clinically significant in relation to increased risk of local recurrence and distant metastases. However, a follow-up analysis (mean observation, 72 months) indicated no prognostic significance in cases with positive EpCAM expression because these patients underwent treatment of radioiodine, which can eliminate microdisseminated tumor residue.18 Nevertheless, detection of EpCAM is clinical significant. The concept is supported by 2 studies that identified the nucleus localization of EpCAM as an aggressive marker for poor prognosis among patients with thyroid cancer.17,32 In our study, the pattern of EpCAM expression showed weak staining in normal human thyroid tissue and strong staining in PTC tissue, with the staining increasing from stage 1 to stage 4. We also found that increased levels of cancer stem cell markers (CD44, CD133, and EpCAM) were positively related to the TNM staging of PTC. However, future studies are needed to define the specificity of cancer stem cell markers in PTC. Conclusions Identification of accurate prognostic molecular markers for papillary thyroid carcinoma (PTC) is important because most patients with PTC may be erroneously diagnosed as having low-risk tumors. Our findings suggest that, compared with traditional histopathological examination, tissue-array based immunohistochemistry for cancer stem cell marker detection can more accurately evaluate the biological characteristics of PTC. Thus, locating cancer stem cells in a subset of patients with PTC may be molecular markers that can be used as prognostic markers and to screen for tumor dissemination. However, whether these cancer stem cells markers are potential therapeutic targets for effective adjuvant treatment strategies requires additional evaluation. References 1. Cancer Facts & Figures 2015. Atlanta, GA: American Cancer Society; 2015. http://www.cancer.org/acs/groups/content/@editorial/documents/document/acspc-044552.pdf. Accessed September 4, 2015. 2. Ahn SH, Henderson YC, Williams MD, et al. Detection of thyroid 514 Cancer Control cancer stem cells in papillary thyroid carcinoma. J Clin Endocrinol Metab. 2014;99(2):536-544. 3. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Thyroid Carcinoma. v2.2015. http://www.nccn. org/professionals/physician_gls/pdf/thyroid.pdf. Accessed September 4, 2015. 4. Guo K, Wang Z. Risk factors influencing the recurrence of papillary thyroid carcinoma: a systematic review and meta-analysis. Int J Clin Exp Pathol. 2014;7(9):5393-5403. 5. Hardin H, Montemayor-Garcia C, Lloyd RV. Thyroid cancer stem-like cells and epithelial-mesenchymal transition in thyroid cancers. Hum Pathol. 2013;44(9):1707-1713. 6. Yun JY, Kim YA, Choe JY, et al. Expression of cancer stem cell markers is more frequent in anaplastic thyroid carcinoma compared to papillary thyroid carcinoma and is related to adverse clinical outcome. J Clin Pathol. 2014;67(2):125-133. 7. Rycaj K, Tang DG. Cell-of-origin of cancer versus cancer stem cells: assays and interpretations. Cancer Res. 2015;75(19):4003-4011. 8. Klonisch T, Hoang-Vu C, Hombach-Klonisch S. Thyroid stem cells and cancer. Thyroid. 2009;19(12):1303-1315. 9. Derwahl M. Linking stem cells to thyroid cancer. J Clin Endocrinol Metab. 2011;96(3):610-613. 10. Fierabracci A. Identifying thyroid stem/progenitor cells: advances and limitations. J Endocrinol. 2012;213(1):1-13. 11. Lin RY. Thyroid cancer stem cells. Nat Rev Endocrinol. 2011;7(10): 609-616. 12. Davies TF, Latif R, Minsky NC, et al. Clinical review: the emerging cell biology of thyroid stem cells. J Clin Endocrinol Metab. 2011;96(9):2692-2702. 13. Hardin H, Guo Z, Shan W, et al. The roles of the epithelial-mesenchymal transition marker PRRX1 and miR-146b-5p in papillary thyroid carcinoma progression. Am J Pathol. 2014;184(8):2342-2354. 14. Tang DG. Understanding cancer stem cell heterogeneity and plasticity. Cell Res. 2012;22(3):457-472. 15. Klonisch T, Wiechec E, Hombach-Klonisch S, et al. Cancer stem cell markers in common cancers - therapeutic implications. Trends Mol Med. 2008;14(10):450-460. 16. Nilubol N, Boufraqech M, Zhang L, et al. Loss of CPSF2 expression is associated with increased thyroid cancer cellular invasion and cancer stem cell population, and more aggressive disease. J Clin Endocrinol Metab. 2014;99(7):E1173-E1182. 17. He HC, Kashat L, Kak I, et al. An Ep-ICD based index is a marker of aggressiveness and poor prognosis in thyroid carcinoma. PLoS One. 2012;7(9):e42893. 18. Rehders A, Anlauf M, Adamowsky I, et al. Is minimal residual lymph node disease in papillary thyroid cancer of prognostic impact? An analysis of the epithelial cell adhesion molecule EpCAM in lymph nodes of 40 pN0 patients. Pathol Oncol Res. 2014;20(1):185-190. 19. Edge SB, Byrd DR, Compton CC, et al, eds. AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer; 2010:87-96. 20. Gires O. Lessons from common markers of tumor-initiating cells in solid cancers. Cell Mol Life Sci. 2011;68(24):4009-4022. 21. Colak S, Medema JP. Cancer stem cells--important players in tumor therapy resistance. FEBS J. 2014;281(21):4779-4791. 22. Figge J, del Rosario AD, Gerasimov G, et al. Preferential expression of the cell adhesion molecule CD44 in papillary thyroid carcinoma. Exp Mol Pathol. 1994;61(13):203-211. 23. Ross JS, del Rosario AD, Sanderson B, et al. Selective expression of CD44 cell-adhesion molecule in thyroid papillary carcinoma fine-needle aspirates. Diagn Cytopathol. 1996;14(4):287-291. 24. Kim JY, Cho H, Rhee BD, et al. Expression of CD44 and cyclin D1 in fine needle aspiration cytology of papillary thyroid carcinoma. Acta Cytol. 2002;46(4):679-683. 25. Ricci-Vitiani L, Lombardi DG, Pilozzi E, et al. Identification and expansion of human colon-cancer-initiating cells. Nature. 2007;445(7123):111-115. 26. Friedman S, Lu M, Schultz A, et al. CD133+ anaplastic thyroid cancer cells initiate tumors in immunodeficient mice and are regulated by thyrotropin. PLoS One. 2009;4(4):e5395. 27. Zito G, Richiusa P, Bommarito A, et al. In vitro identification and characterization of CD133(pos) cancer stem-like cells in anaplastic thyroid carcinoma cell lines. PLoS One. 2008;3(10):e3544. 28. Liu J, Brown RE. Immunohistochemical detection of epithelial-mesenchymal transition associated with stemness phenotype in anaplastic thyroid carcinoma. Int J Clin Exp Pathol. 2010;3(8):755-762. 29. Parkin DM, Bray F, Ferlay J, Pisani P. Global cancer statistics, 2002. CA Cancer J. Clin. 2005; 55: 74-108. 30. Went P, Vasei M, Bubendorf L, et al. Frequent high-level expression of the immunotherapeutic target Ep-CAM in colon, stomach, prostate and lung cancers. Br J Cancer. 2006;94(1):128-135. 31. Schmelzer E, Reid LM. EpCAM expression in normal, non-pathological tissues. Front Biosci. 2008;13:3096-3100. 32. Ralhan R, Cao J, Lim T, et al. EpCAM nuclear localization identifies aggressive thyroid cancer and is a marker for poor prognosis. BMC Cancer. 2010;10:331. October 2015, Vol. 22, No. 4 Special Report Should Vital Signs Be Routinely Obtained Prior to Intravenous Chemotherapy? Results From a 2-Center Study Smitha Menon, MD, Nathan R. Foster, MS, Sherry Looker, RN, Kristine Sorgatz, RN, Pashtoon Murtaza Kasi, MBBS, Robert R. McWilliams, MD, and Aminah Jatoi, MD Background: The American Society of Clinical Oncology and the Oncology Nursing Society have issued guidelines stating that the vital signs of patients should be routinely checked on days that intravenous chemotherapy is administered. This study sought evidence to justify this approach. Methods: This trial focused on consecutive patients with cancer from 2 institutions and evaluated outcomes during the first cycle of gemcitabine-based chemotherapy. The primary end point of the study was a visit to the ED, hospitalization, or death during the first cycle of chemotherapy. Results: Medical records from 1,158 patients were reviewed, and vital signs were checked in 589 patients on day 1 and in 486 on day 8. A total of 148 patients (12.8%) were evaluated in the emergency department (ED), 145 (12.5%) were hospitalized, and 11 (0.9%) died during their first cycle of chemotherapy. In multivariate analyses, which were adjusted for age, sex, cancer type, role of chemotherapy, number of chemotherapy drugs administered on day 1, and institution, checking vital signs on day 1 was associated with neither higher rates of ED visits nor with increased hospitalization; however, checking vital signs on day 8 was associated with higher rates of ED visits (odds ratio [OR]: 3.71; 95% confidence interval [CI]: 2.18–6.22; P < .0001) and higher rates of hospitalizations (OR: 3.98; 95% CI: 2.34–6.73; P < .0001). Conclusion: This study suggests a need for additional, evidence-based data to support the routine checking of vital signs prior to administering cancer chemotherapy. Introduction The American Society of Clinical Oncology and the Oncology Nursing Society have issued joint guidelines stating that the vital signs (blood pressure, pulse, respiratory rate, and temperature) of patients with cancer should be routinely and consistently checked on the day that intravenous chemotherapy is administered.1 Yet, to our knowledge, data to justify — or refute — this recommendation are lacking. At least 2 factors point to the need to investigate the value of checking vital signs prior to the administration of intravenous chemotherapy in patients with cancer. In the nononcological setting, this issue is an important focus of ongoing research. Storm-Versloot et al2 performed a meta-analysis of the routine monitoring of vital signs in hospitalized patients and observed that, among 15 studies (N = 42,565 patients), conclusions were highly mixed. The investigators noted that studies appeared From the Division of Hematology/Oncology, Medical College of Wisconsin, Milwaukee, Wisconsin (SM), and the Divisions of Biomedical Statistics (NRF) and Medical Oncology (SL, KS, PMK, RRM, AJ), Mayo Clinic, Rochester, Minnesota. Submitted July 20, 2015; accepted July 29, 2015. Address correspondence to Aminah Jatoi, MD, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55904. E-mail: Jatoi.Aminah@mayo.edu No significant relationships exist between the other authors and the companies/organizations whose products or services may be referenced in this article. October 2015, Vol. 22, No. 4 flawed and suggested a need for rigorous research “specifically intended to investigate the clinical relevance of routinely measured vital signs.”2 In addition, health care professionals are beginning to question the role of routinely checking vital signs of patients in nononcology settings, a finding that suggests similar questioning should occur in oncology settings.3-5 In view of a paucity of data on this topic in the oncology setting, the need to establish a role for the routine checking of vital signs prior to the administration of chemotherapy is important. Second, checking vital signs prior to the administration of chemotherapy has enormous, onerous implications. If one assumes that checking vital signs takes about 5 minutes and if one works in a chemotherapy unit that treats approximately 150 patients per day, as is currently the case at the Mayo Clinic (Rochester, Minnesota), checking vital signs requires at least 1 full-time staff member whose only task is to check patient vital signs. Thus, acquiring evidence to support or refute the importance of this practice offers more than academic interest and is in keeping with Elshaug et al,6 who describe that a “groundswell of activity is seeking to identify and reduce the use of health care service that provide little or no benefit — whether through overuse or misuse.” Thus, the primary goal of this 2-institution study was to generate and analyze data to better understand whether the routine checking of vital signs prior to the administration of intravenous chemotherapy is Cancer Control 515 associated with fewer complications — specifically, with lower rates of emergency department (ED) visits and hospitalizations. Methods Overview This study was undertaken at the Medical College of Wisconsin (Milwaukee, Wisconsin) and the Mayo Clinic. The Institutional Review Board of each institution reviewed and approved the study protocol prior to data acquisition and analyses. These institutions were chosen because, historically, one routinely checked vital signs prior to the administration of chemotherapy (the former) and the other did not. An ED visit, hospitalization, or death during the first cycle of chemotherapy was considered a complication, which was the primary end point of the study. Routine Nursing Assessment Both institutions incorporated routine clinical assessment prior to the administration of chemotherapy on days when a prescribing health care professional had not seen the patient. This prechemotherapy nursing assessment consisted of asking patients about their general well-being and about specific signs and symptoms such as fever, fatigue, nausea, and vomiting. Concerns raised during the nursing assessment could conceivably trigger a vital-sign check based on clinical judgment and the temporal incidence and severity of the signs or symptoms. Rationale for Focusing on Patients Treated With Gemcitabine This study focused on patients with cancer about to receive their first cycle of gemcitabine-based chemotherapy. Pharmacy records were used to identify patients. The rationale for focusing on gemcitabine-treated patients is as follows: (1) gemcitabine is typically administered on day 8 in the chemotherapy cycle, and this day often does not entail a visit with a prescribing health care professional who likely would have checked vital signs as part of a routine physical examination, (2) this chemotherapy agent is used to treat a variety of cancers, leading to greater generalizability of findings, and (3) the adverse events of this agent are characteristic of those observed with classical, conventional chemotherapy (nausea, vomiting, myelosuppression, diarrhea), a profile that leads to greater generalizability of findings. We decided to focus on the first cycle of gemcitabine-based chemotherapy alone because the risk of adverse events generally increases with subsequent cycles, and this restriction would potentially enhance the homogeneity of the study population. Data Abstraction Consecutive medical records of patients with cancer 516 Cancer Control treated with gemcitabine were reviewed, starting with the most recent and proceeding back in time. The following information was abstracted: patient date of birth, sex, date of administration of the first cycle of gemcitabine-based chemotherapy, cancer type, whether chemotherapy was given for metastatic disease or as adjuvant treatment, number of drugs administered within the regimen, institution of chemotherapy administration, whether vital signs were checked on days 1 and 8 of the chemotherapy cycle (these data were gathered irrespective of which health care professional performed the check), ED visit or hospitalization during the first chemotherapy cycle, and death during the first chemotherapy cycle. Sample Size and Analyses In calculating sample size, the study team estimated that approximately one-third of study patients would have had their vital signs routinely checked based on the anticipated patient representation from the Medical College of Wisconsin. Based on prior reported rates of adverse events in clinical trials, 5% of patients who had their vital signs checked were estimated to have a major complication during their first dose of gemcitabine-based chemotherapy. We estimated that this rate would increase to 10% in the absence of checking vital signs; this 5% effect size was derived from other clinical settings in which this increased complication rate approximates the upper limit of acceptability.7-9 We estimated that a target sample size of 1,015 study patients would enable us to detect the above with 80% power, assuming a 2-sided significance level of .05. Analyses consisted of direct comparisons of complication rates between study patients who did and did not have their vital signs checked at specific time points. If chemotherapy was not administered on a particular day, then the complication data from that study patient were still included in the analyses relevant to that day. For univariate and multivariate analyses, logistical regression models were constructed to determine which variables were associated with ED visits and hospitalizations (in the event of a very low complication rate [eg, low death rate], this analysis was not performed). A 2-sided P value of less than .05 was considered statistically significant. Statistical analyses were performed using SAS version 9.3 (SAS Institute, Cary, North Carolina) and JMP software (SAS Institute). Results Patient Demographics We reviewed the medical records of 1,158 study patients. Vital signs were checked for 589 study patients on day 1 and for 486 study patients on day 8. Based on whether vital signs had been checked, patient characteristics were similar, although cancer type, number of drugs administered, and whether patients received October 2015, Vol. 22, No. 4 adjuvant treatment or treatment for metastatic cancer differed between groups, at times, based on vital-sign assessment and institution (Table 1). Complications and Vital-Sign Checks A total of 148 study patients (12.8%) were evaluated in the ED, 145 (12.5%) were hospitalized, and 11 (0.9%) died during their first cycle of chemotherapy. Subgroup analyses found no statistically significant differences in these complication rates based on institution, except for a trend toward a greater number of ED visits in patients from the Medical College of Wisconsin: 60 visits (15%) compared with 88 visits (12%; P = .08). Checking vital signs on day 1 was associated with a trend toward more ED visits (85 visits [15%]) compared with patients whose vitals signs were not checked (63 [11%]; P = .09). By contrast, hospitalizations and deaths were not statistically different based on day 1 vital sign checks. Checking vital signs on Table 1. — Patient Demographics Characteristic Institution Wisconsina Median age, y (range) 62 (42–91) Mayo Day 1 Vital Signs Checked? P value 63 (22–95) No (N = 5690) Yes (N = 589) 63 (22–95) 62 (42–91) .67 P value Day 8 Vital Signs Checked? No (N = 670) Yes (N = 486) 62 (22– 95) 62 (42– 91) .02 P value .35 Sex, n (%) Male Female 204 (52) 192 (49) 369 (48) 393 (52) 283 (50) 286 (50) 290 (49) 299 (51) .32 325 (49) 345 (52) 247 (51) 239 (49) .86 .44 Cancer type, n (%) Pancreas Lung Breast Gynecological Genitourinary Other 73 (19) 59 (15) 17 (4) 38 (10) 99 (25) 109 (28) 89 (12) 141 (19) 47 (6) 115 (15) 166 (22) 204 (27) 61 (11) 102 (18) 31 (5) 76 (13) 146 (26) 153 (27) 74 (11) 121 (18) 41 (6) 102 (15) 156 (23) 176 (26) 101 (17) 98 (17) 33 (6) 77 (13) 119 (20) 160 (27) .002 88 (18) 79 (16) 23 (5) 51 (11) 109 (23) 135 (28) .03 .005 Chemotherapy, n (%) Adjuvant Metastatic Unspecified 38 (10) 316 (80) 39 (10) 72 (9) 658 (86) 32 (4) 56 (10) 486 (85) 27 (5) 54 (9) 488 (83) 44 (8) .0006 65 (10) 576 (86) 29 (4) 45 (9) 397 (82) 42 (9) .14 .01 Drugs given on day 1, n (%) 1 2 3 4 76 (19) 320 (81) 0 0 196 (26) 499 (66) 65 (9) 2 (0) 143 (25) 384 (68) 41 (7) 1 (0) 129 (22) 435 (74) 24 (4) 1 (0) < .0001 173 (26) 448 (67) 48 (7) 1 (0) 99 (20) 369 (76) 17 (4) 1 (0) .09 .008 Drugs given on day 8, n (%) 0 1 2 3 74 (19) 160 (40) 162 (41) 0 150 (20) 380 (50) 220 (29) 12 (2) 111 (20) 288 (51) 165 (29) 5 (1) < .0001 a 113 (19) 252 (43) 217 (37) 7 (1) 126 (19) 353 (53) 186 (28) 5 (1) .02 96 (20) 187 (39) 196 (40) 7 (1) < .0001 Percentages do not always sum to 100% because of rounding or missing data. October 2015, Vol. 22, No. 4 Cancer Control 517 day 8 was associated with more patients visiting an ED (85 [18%] compared with 62 [9%]; P < .0001) and more patients being hospitalized (81 [17%] compared with 63 [9%]; P = .0002). Multivariate logistic regression analyses are shown in Table 2 and show that checking vital signs on day 1 was neither associated with a higher rate of ED visits nor an increased rate of hospitalization. However, checking vital signs was associated with a higher rate of ED visits and higher hospitalization rates when checked on day 8. Discussion This study was undertaken to assess whether or not checking vital signs on the day of chemotherapy administration is associated with higher complication rates (eg, ED visits, hospitalizations). Our study findings indicate this was not the case. Rather, the opposite was found: Checking vital signs on the day of chemotherapy administration appears to be associated with higher rates of ED visits and hospitalizations. However, these findings do not indicate that checking vital signs leads to higher rates of complications; rather, patients who appeared ill presumably had their vital signs checked only because they appeared ill; these patients were subsequently referred to the ED or were hospitalized. Thus, the findings of this study suggest that not checking vital signs routinely prior to the administration of intravenous chemotherapy does not have adverse consequences. The role of routinely checking vital signs is also being questioned in other areas of clinical medicine. Historically, checking vital signs was an essential, mandatory component of patient care. However, more recent studies — some of which include the routine monitoring of vital signs in hospitalized patients — have questioned the value of this practice and suggest that patients present with other symptoms or signs well before they manifest a concerning change in their vital signs.2,3 In hospital settings, the concern for compromising patient sleep, as well as escalating health care costs, has also led to the omission or curtailing of routine vital sign checks.4,5 Although the role of routinely checking vital signs continues to remain controversial, emerging trends show that this practice has been discontinued in some circumstances.10,11 A critical distinction should be made between routinely checking vital signs and checking vital signs when patients appear ill or when patients are receiving antineoplastic agents that put them at risk for hypertension. Patients who fall within these latter 2 categories should continue to have vital signs checked. We acknowledge that patients with cancer who are ill or asymptomatic but who are receiving a medication such as bevacizumab should have at least a partial set of vital signs obtained. 518 Cancer Control Table 2. — Multivariate Logistic Regression Model End Point Checking Vital Signs OR (95% CI)a P value ED visits Day 1 (yes vs no) 1.2 (0.72–1.97) .48 Day 8 (yes vs no) 3.71 (2.18–6.22) < .0001 Day 1 (yes vs no) 1.32 (0.80–2.14) .27 Day 8 (yes vs no) 3.98 (2.34–6.73) < .0001 Hospitalizations a Analyses were adjusted for adjusted for age, sex, cancer type, chemotherapy type (adjuvant vs for metastatic disease), and number of drugs prescribed on day 1 or 8 (day 1 for the day 1 vital signs predictor and day 8 for the day 8 vital signs predictor), and institution. CI = confidence interval, ED = emergency department, OR = odds ratio. Limitations The retrospective nature of this study makes it impossible to determine why the checking of vital signs occurred or did not outside the routine practice of each specific institution. On day 1, or close to day 1, patients are likely to see their health care professional, who has performed an in-depth assessment of their status. Therefore, one might suggest that nurses can forgo checking vital signs if a physician had seen the patient at some very recent time point. Furthermore, patients with abnormal vital signs during their visit with their oncologist might have been deflected from receiving chemotherapy, resulting in a bias toward seeing normal vital signs on day 1 prior to administering chemotherapy. Of note, the foregoing is speculative, thus emphasizing the limitations of the design used in this study. This study might have been underpowered with respect to determining whether early interventions, such as hospitalization, based on abnormal vital signs would have prevented more serious complications. We were unable to derive such information from a retrospective study. In addition, the data from the current study are not derived from a randomized trial. As a result, comparative participant groups might be imbalanced with respect to institutional practices that could have influenced complication rates or patient predisposition for chemotherapy toxicity. If a prospective trial were to be performed, then the focus of that study should be on asymptomatic patients alone, with the exclusion of those receiving chemotherapy agents that might increase blood pressure. It would also be important to balance study arms based on the extent of prior cancer therapy and other factors that tend to lead to greater chemotherapy toxicity. One might argue that the great challenges of performing such a trial add to the importance of the data presented in this paper. October 2015, Vol. 22, No. 4 Conclusion Our findings question whether the absence of evidence to support the checking of vital signs should lead to the conclusion that vital signs should be obtained. At the very least, these study results call for further evidence-based data to assess this routine, expense-generating clinical practice. References 1. Neuss MN, Polovich M, McNiff K, et al. 2013 updated American Society of Clinical Oncology/Oncology Nursing Society chemotherapy administration safety standards including standards for the safe administration and management of oral chemotherapy. J Oncol Pract. 2013;9(2 suppl):5s-13s. 2. Storm-Versloot MN, Verweij L, Lucas C, et al. Clinical relevance of routinely measured vital signs in hospitalized patients: a systematic review. J Nurs Scholarsh. 2014;46(1):39-49. 3. Evans D, Hodgkinson B, Berry J. Vital signs in hospital patients: a systematic review. Int J Nurs Stud. 2001;38(6):643-650. 4. Fukayama H, Yagiela JA. Monitoring of vital signs during dental care. Int Dent J. 2006;56(2):102-108. 5. Leinonen T, Leino-Kilpi H. Research in peri-operative nursing care. J Clin Nurs. 1999;8(2):123-138. 6. Elshaug AG, McWilliams JM, Landon BE. The value of low-value lists. JAMA. 2013 Feb 27;309(8):775-6. 7. van Hagen P, Hulshof MC, van Lanschot JJ, et al. Preoperative chemoradiotherapy for esophageal or junctional cancer. N Engl J Med. 2012;366(22):2074-2084. 8. Attal M, Harousseau JL, Stoppa AM, et al. A prospective, randomized trial of autologous bone marrow transplantation and chemotherapy in multiple myeloma. Intergroupe Français du Myélome. N Engl J Med. 1996;335(2):91-97. 9. Child JA, Morgan GJ, Davies FE, et al. High-dose chemotherapy with hematopoietic stem-cell rescue for multiple myeloma. N Engl J Med. 2003;348(19):1875-1883. 10. Pellicane AJ. Relationship between nighttime vital sign assessments and acute care transfers in the rehabilitation inpatient. Rehabil Nurs. 2014;39(6):305-310. 11. Bartick MC, Thai X, Schmidt T, et al. Decrease in as-needed sedative use by limiting nighttime sleep disruptions from hospital staff. J Hosp Med. 2010;5(3):E20-E24. October 2015, Vol. 22, No. 4 Cancer Control 519 Special Report Advancing Cancer Control Through Research and Cancer Registry Collaborations in the Caribbean Rishika Banydeen, MPH, Angela M.C. Rose, MSc, Damali Martin, PhD, William Aiken, MD, Cheryl Alexis, MBBS, MSc, MRCP, Glennis Andall-Brereton, PhD, Kimlin Ashing, PhD, J. Gordon Avery, MB, ChB, MD, Penny Avery, SRN, Jacqueline Deloumeaux, MD, PhD, Natasha Ekomaye, Owen Gabriel, MD, Trevor Hassell, MBBS, Lowell Hughes, MBBS, Maisha Hutton, MSc, Shravana Kumar Jyoti, MD, Penelope Layne, RN/RM, Danièle Luce, PhD, Alan Patrick, MD, Patsy Prussia, MBBS, Juliette Smith-Ravin, PhD, Jacqueline Veronique-Baudin, PhD, Elizabeth Blackman, MPH, Veronica Roach, SRN, and Camille Ragin, PhD Background: Few national registries exist in the Caribbean, resulting in limited cancer statistics being available for the region. Therefore, estimates are frequently based on the extrapolation of mortality data submitted to the World Health Organization. Thus, regional cancer surveillance and research need promoting, and their synergy must be strengthened. However, differences between countries outweigh similarities, hampering registration and availability of data. Methods: The African-Caribbean Cancer Consortium (AC3) is a broad-based resource for education, training, and research on all aspects of cancer in populations of African descent. The AC3 focuses on capacity building in cancer registration in the Caribbean through special topics, training sessions, and biannual meetings. We review the results from selected AC3 workshops, including an inventory of established cancer registries in the Caribbean region, current cancer surveillance statistics, and a review of data quality. We then describe the potential for cancer research surveillance activities and the role of policymakers. Results: Twelve of 30 Caribbean nations have cancer registries. Four of these nations provide high-quality incidence data, thus covering 14.4% of the population; therefore, regional estimates are challenging. Existing research and registry collaborations must pave the way and are facilitated by organizations like the AC3. Conclusions: Improved coverage for cancer registrations could help advance health policy through targeted research. Capacity building, resource optimization, collaboration, and communication between cancer surveillance and research teams are key to obtaining robust and complete data in the Caribbean. Introduction Cancer is increasing in frequency and is a leading cause of death worldwide. As such, it is a principal priority for health authorities of many countries.1 An estimated 14 million new cancer cases occurred in the world in 2012.2 Lung, female breast, colorectal, pros- From the Clinical Research and Innovation Unit (RB) and Martinique Cancer Registry (JS-R, JV-B), University Hospital of Martinique, Fort-de-France, Martinique, French West Indies, Chronic Disease Research Centre (AMCR, NE, PP) and Faculty of Medical Sciences (CA), University of the West Indies, St Michael, Barbados, West Indies, Epidemiology and Genomics Research Program, Division of Cancer Control and Population Sciences, National Cancer Institute, National Institute of Health, Bethesda, Maryland (DM), Departments of Surgery, Radiology, Anaesthesia, and Intensive Care, University of the West Indies, Mona, Kingston, Jamaica, West Indies (WA), Surveillance, Disease Prevention and Control Division, Caribbean Public Health Agency, Port of Spain, Trinidad, West Indies (GA-B), Center of Community Alliance for Research and Education, Department of Population Science, City of Hope Medical Center, Duarte, California (KA), University of Medicine and Health Sciences, St Kitts (JGA), and Pink Lily Cancer Care, Nevis Maternal Health Fund (PA), St Kitts and Nevis, West Indies (JGA), Guadeloupe Cancer Registry, University Hospital of Pointe-à-Pitre, Guadeloupe, West Indies (JD), Oncology Department, Victoria Hospital, Castries Saint Lucia (OG), Healthy Caribbean Coalition, Barbados, West Indies (TH, MH), Hughes Medical Centre, Anguilla, British West Indies (LH), Health Service Authority Hospital, Georgetown, Cayman Islands, British West Indies (SKJ), Guyana Cancer Registry, Ministry of Health, Queenstown, Guyana (PL), French National Institute of Health and Medical Research, Guadeloupe, French West Indies (DL), Tobago Health Studies Office, Scarborough, Trinidad and Tobago (AP), Cancer Prevention and Control Program, Fox Chase Cancer Center, Temple Health, Philadelphia, Pennsylvania (EB, CR), Dr Elizabeth Quamina National Cancer Registry of Trinidad and Tobago, Eric Williams Medical Sciences Complex, Mount Hope, Trinidad and Tobago (VR). Mrs Banydeen, Rose, Layne, and Roach and Drs Aiken, Ashing, Avery, Deloumeaux, Jyoti, Luce, Patrick, Smith-Ravin, Veronique-Baudin, and Ragin are members of the African-Caribbean Cancer Consortium. This work is supported in part by the National Cancer Institute (P30 CA006927) and (R13 CA192672-03 to C.R.). No significant relationships exist between the other authors and the companies/ organizations whose products or services may be referenced in this article. Submitted December 8, 2014; accepted July 17, 2015. Address correspondence to Camille Ragin, PhD, Cancer Prevention and Control Program, Fox Chase Cancer Center, 333 Cottman Avenue, Philadelphia, PA 19111. E-mail: camille.ragin@fccc.edu 520 Cancer Control October 2015, Vol. 22, No. 4 tate, and stomach cancers accounted for nearly 50% of all cases diagnosed and of cancer-related deaths worldwide.2 The Caribbean, whose location is detailed in Fig 1, is no exception, with malignant neoplasms being the leading cause of mortality for those living in the Caribbean.3 Although Caribbean nations share the same urgency in the fight against cancer in terms of surveillance and research, important limitations exist in addressing this issue.4,5 For example, although different countries share many similarities in terms of environmental, ethnic predominance (most Englishspeaking Caribbean countries comprise majority African-origin populations), and other common risk factors, geographical, demographic, and ethnic differences are also present.6 Not all Caribbean countries are islands, and their populations vary in size: from more than 11 million in Cuba to about 5,000 for Montserrat.7 Some countries have majority populations of East Indian ethnic origin, and many have an increasing proportion of their population of mixed ethnic background.8 Economic differences also exist, with some Ca- ribbean countries classified as high- (eg, Barbados, Bahamas), upper-middle (eg, Jamaica, Suriname), lower-middle (eg, Guyana), or low-income economies (eg, Haiti).9 Although ethnic and environmental factors influence trends in rates of cancer incidence and mortality, such factors are generally correlated with economic development.10,11 Variation in economic development throughout the Caribbean also means a range of access to and quality of health care across different countries; in some communities, insufficient or inadequate health insurance, or geographical remoteness, or estrangement from adequate specialized health infrastructure has resulted in a higher frequency of cancers with poor prognoses (due to diagnosis and treatment at advanced stages) and more deaths.12 It is necessary to monitor epidemiological trends in cancer (eg, through surveillance), particularly in low-resource countries undergoing demographic transition with increasingly aging populations like those in the Caribbean.6,13 This is even more important because high-quality data on cancer are lacking from low- and medium-resource countries, and, con- Fig 1. — Map of the Caribbean. October 2015, Vol. 22, No. 4 Cancer Control 521 sidering the differences between countries across the Caribbean, generalizations have been difficult.10 In addition, research is vital for understanding the specific determinants of most cancers, especially those affecting high-risk populations such as African Caribbean or indigenous populations, such as Amerindians, and to provide better guidance and evaluation of cancer control policies to develop targeted and efficient programs for cancer prevention and care.6,14,15 Although the distinction between surveillance and research is important, the synergy between them is often overlooked. Surveillance is necessary for continued disease monitoring, and its data analysis may hint at previously unknown insights. By contrast, the value of research lies in the ability of researchers to examine what can only be alluded to, but not proved, using surveillance data. Therefore, surveillance is often termed hypothesis generating and research hypothesis testing. Collaboration between researchers using these different methods for generating information on a subject can be powerful, although often they run along parallel paths.16 However, considering the intra-Caribbean variation, countries in the region cannot be “equal in arms” against the cancer epidemic. Not all Caribbean countries possess sufficient essential health surveillance infrastructure to provide efficient cancer registration and quality cancer data. Where established and efficient cancer surveillance is present, little optimization of cancer registration exists to help make progress through cancer research. In addition, knowledge, resources, and collaboration may be too limited to utilize and address research needs overall, including via cancer registries.14 Several prior regional scientific workshops and conferences have identified the few existing cancer surveillance systems of the Caribbean and have highlighted the ongoing scientific collaborations implicating cancer registries.17-19 These workshops advocate for more dynamic and productive communication between relevant partners while also emphasizing the growing importance of, and need for, cancer registration to address the increasing cancer burden through targeted research.17-19 Herein we describe the latest 2013 and 2014 workshops coordinated by the African-Caribbean Cancer Consortium (AC3), which facilitates and promotes the collaboration between cancer research and surveillance. We also join the AC3 and other concerned cancer surveillance and research stakeholders in the Caribbean in calling for the reinforcement of cancer registration in the Caribbean to expand population-based coverage by cancer registries and obtain more complete and reliable data for their strategic use in guiding requisite cancer control interventions and collaborative research. 522 Cancer Control Caribbean Nations The Caribbean region refers to the 15 Caribbean nation members and 5 associate members of the Caribbean Community (CARICOM), in addition to 10 other Caribbean nations (French Martinique, Guadeloupe, and French Guiana, as well as other Caribbean islands of the Greater and Lesser Antilles; see Fig 1). 20 The 30 Caribbean nations referred to in this paper include Anguilla, Antigua and Barbuda, the Bahamas, Barbados, Belize, Bermuda, the British Virgin Islands, the Cayman Islands, Cuba, Dominica, the Dominican Republic, French Guiana, Grenada, Guadeloupe, Guyana, Haiti, Jamaica, Martinique, Montserrat, The Netherlands Antilles (Aruba, Bonaire, Curaçao, Saba, St Eustatius, and the Dutch half of St Martin and St Maarten), Puerto Rico, St Barthélemy, St Kitts and Nevis, St Lucia, St Martin (the French half of St Martin and St Maarten), St Vincent and the Grenadines, Suriname, Trinidad and Tobago, the Turks and Caicos, and the US Virgin Islands. Although they predominantly comprise islands in the Caribbean Sea for which the official language is English, notable exceptions exist. French Guiana, Suriname and Guyana are on mainland South America while Belize is on mainland Central America; French Guiana, Haiti, Martinique, Guadeloupe, Saint Barthélemy and Saint Martin are French-speaking, while the official language of Suriname, Aruba, Bonaire, Curaçao and Sint Maarten is Dutch, and that of Cuba, the Dominican Republic, and Puerto Rico is Spanish. African-Caribbean Cancer Consortium The mission of the AC3 is to study viral, genetic, environmental, and lifestyle factors for cancer risk and outcomes in populations of African descent.21 The consortium is part of the Epidemiology and Genomics Research Program consortia of the National Cancer Institute (NCI), which is composed of 3 connected networks of investigators in the United States, Africa, and the Caribbean region.21 The AC3 has 120 members and is a broad-based resource for education, training, and research on the etiology, screening, prevention, treatment, and survivorship related to cancer in populations of African descent.21 Within the AC3, 7 Caribbean cancer registries are represented (5 are national: Barbados, Guyana, Guadeloupe, Martinique, and Trinidad and Tobago; 2 are subnational: Cayman Islands and Jamaica).4,17,18,21 Capacity-Building Activities The AC3 focuses on capacity-building efforts in the field of cancer registration in the Caribbean by hosting a series of special topics and training sessions during biannual scientific meetings and other supporting October 2015, Vol. 22, No. 4 initiatives.4,18,19 Five scientific meetings have been conducted to date since 2007. During the 2007 and 2008 AC3 meetings, presentations by cancer registrars provided an overview of the cancer burden in the Caribbean region.17,18 During the 2010 meeting, rationale for cancer registration, the different types of cancer registries, and the relevant steps for planning and implementation of a cancer registry were discussed.4 The 2012 meeting provided another opportunity for presentations of national cancer registry data from 4 cancer registries in lower–middle-income countries (Kenya, Guyana, Jamaica, and Trinidad and Tobago).5 Cancer statistics were presented that revealed the reality of cancer burden in these Caribbean countries.5 Cancer registration activities concluded with a satellite meeting with the NCI.5 To continue what the AC3 has accomplished, the information learned thus far, and to address current needs, an AC3 cancer registry workshop was organized in 2013 as a preconference to the annual meeting of the Caribbean Public Health Agency (CARPHA).22,23 The primary purpose of the 1-day meeting was to amplify, promote, and sustain Caribbean scientific research through collaborations with cancer registries.22 The objectives of the workshop were to describe the applications and utilization of cancer registry data in collaborative research and to discuss the importance of policy decision-making and its impact on existing or developing national cancer registries and cancer control.22 The workshop united existing and aspiring cancer registrars, pathologists, clinical and biomedical scientists, representatives of public health organizations, and cancer advocates.22 It also presented opportunities for dialogue and collaboration between English- and French-speaking Caribbean nations.22 This initiative was further reinforced by the 2014 Cancer Surveillance in the Caribbean meeting, a joint effort of several stakeholders that included the NCI, the CARPHA, the US Centers for Disease Control and Prevention, the International Agency for Research on Cancer (IARC), the Pan American Health Organization (PAHO), the US Northern Command, and the AC3.24 As of this publication, the AC3 2014 scientific meeting was the most recent gathering to discuss strategies in the way forward toward better cancer registration and collaborative research for the Caribbean region.21,25 Established Cancer Registries Of the 30 nations and territories in the Caribbean region, 12 have established cancer registries, 6 of which have existed for more than 30 years (Puerto Rico, Jamaica-Kingston, Cuba, The Netherlands Antilles, Bermuda, and Martinique) and 1 for 20 years (Trinidad and Tobago), whereas all others have been in operation for 5 to 15 years (Table 1). Of these, 4 cancer regisOctober 2015, Vol. 22, No. 4 Table 1. — Inventory of Established Cancer Registries in the Caribbean Country Type of Registry Year of Establishment National 1951 3,688,000 Urban 1958 1,453,248 Cuba National 1964 11,266,000 Netherlands Antillesb National 1977 199,929 Bermudac National 1979 69,000 Martinique National 1983 404,000 Trinidad and Tobago National 1994 1,341,000 Guyana National 2000 800,000 French Guiana National 2005 249,000 Guadeloupe National 2008 466,000 Barbados National 2010 289,000 Cayman Islands National 2010 54,000 Puerto Rico Jamaica a Estimated Midyear Population12 aThis registration occurs in urban areas alone (Kingston and St Andrew). bIncludes 6 Dutch-Caribbean islands: Curacao, Aruba, Bonaire, Saba, St Eustatius, and St Maarten. restructuring in 2004 and relaunched in 2008. cUnderwent tries (Cuba, Jamaica–Kingston, Martinique, and Puerto Rico), representing 14.4% of the regional population, have contributed high-quality incidence data published by the World Health Organization (WHO).26 Based on discussions from the workshop, emerging cancer registries in other countries (eg, Anguilla, St Kitts and Nevis, St Lucia, the Bahamas and the Cayman Islands) may be hampered by challenges, including limited training opportunities, limited staff availability and qualifications, inadequate information technology support, and transportation difficulties for data retrieval. Other challenges include lack of diagnostic facilities, lack of pathologists, and long reporting delays compounded by poor verification of data, lack of formal death certification, and lack of support from ministries of health. The latter is critical to ensure sustainability of cancer registries and their role in developing and evaluating cancer control strategies.27-29 Surveillance Estimates and Quality of Data The Caribbean region ranks fifth worldwide for cancer incidence and fourth for mortality (185.4 and 102.0 per 100,000 per year, respectively; Fig 2).2 However, disparities exist in the quality of available cancer data, because 4 Caribbean nations have high-quality national or regional cancer incidence data, whereas 2 nations have complete, high-quality cancer mortality data (Table 2).2 Furthermore, for countries without existing Cancer Control 523 Rates, ASR( W ) (per 100,000) Table 2. — Quality of Cancer Data in the Caribbean 350 Availability 300 Incidence Data N High-quality national or regional data 4 Cuba Martinique Puerto Rico Jamaica National data (rates) 1 Trinidad and Tobago Regional data (rates) 0 — Frequency data 0 — No data a 9 Bahamas Barbados Belize Dominican Republic Guadeloupe French Guiana Guyana Haiti Suriname High-quality complete vital registration 2 Bahamas Cuba Medium-quality complete vital registration 8 Barbados Belize Guyana Guadeloupe Martinique French Guiana Puerto Rico Trinidad and Tobago Low-quality complete vital registration 4 Dominican Republic Haiti Jamaica Suriname Incomplete or sample vital registration 0 — Other sourcesb 0 — No data 0 — 250 200 150 100 50 meric a Nor th A Oceania Eur o p e a Americ South e an A sia Caribb Centr a l A m er Afric a ic a 0 Incidence Mortality Data Mortality Fig 2. — ASR(W) of and mortality from all cancer sites per 100,000 per year by world region. The Caribbean region is defined as the Bahamas, Barbados, Cuba, Dominican Republic, Guadeloupe, Martinique, Haiti, Jamaica, Puerto Rico, and Trinidad and Tobago. ASR(W) = world age-standardized incidence rate. Data from reference 2. cancer registries or those with newly established registries, cancer statistics are typically derived from estimates of the WHO compiled from a combination of nationally reported mortality data and hospital-based data.2 Therefore, it is possible that under-reporting is occurring for some Caribbean nations, thus underlying the need for indigenous cancer registry data to accurately reflect the burden of cancer in the region.2 Available Research Data Deeper insight into cancer registration in the Caribbean and its potential perspectives was achieved through a description of available data provided by countries to CARPHA. Highlights from these data included the high prevalence of smoking in some countries (although the general belief is that smoking has a very low prevalence throughout the Caribbean) and a high prevalence of alcohol intake.30,31 Taken altogether, the data suggest an increased risk for the development of noncommunicable diseases, including cancer, with these conditions representing the top 10 causes of death during the last decade in the Caribbean. Although some research on environmental carcinogens has been documented, such as the prostate case-control study in Guadeloupe, the region still lacks research 524 Cancer Control Caribbean Country Not all cancer data originating from registries and other sources in some Caribbean islands have been referenced and evaluated by the International Agency for Research on Cancer. b Cancer registries, verbal autopsy, and surveys, among others. Data from reference 2. a in this domain.32 Other gaps in the data include cancer morbidity and survival rates, clinical management, and population screening rates for specific cancers (cervical, breast, and colon). Usefulness of Cancer Surveillance Data in Research Collaborations The usefulness of cancer registries in collaborating with research for population-based case-control studOctober 2015, Vol. 22, No. 4 ies was also described (Fig 3). For example, data from registries allow researchers to plan or estimate the number of expected cases, identify the main hospitals where cases may be found, or identify patients for recruitment into research studies. During the study, data can be cross-checked with the registry to ensure coverage. Registry data are also useful for checking on selection bias in research studies. If the registry also conducts follow-up for cases, then it can provide the study with additional data on outcomes. Examples of such collaborative work were illustrated by an ongoing study investigating socioeconomic inequalities in cancer incidence and mortality in Martinique and Guadeloupe as well as AC3 collaborations involving the cancer registries in Trinidad and Tobago and Guyana.33 Analyses of data, issuing from these AC3 collaborations, have been conducted or are near completion for Research capacity-Building collaboration Outcomes • Number of expected cases • Case ascertainment (identifying patients for study recruitment) • Selection bias • Geographical variation • Quality of care • Quality of life • Barriers to screening or treatment • Comorbidities and survivorship • Study of risk factors/ specific determinants • Identifying vulnerable/ at-risk groups • Cancer control and management • Cancer outcomes • Public health interventions • Improve quality of diagnosis, survivorship, treatment, and follow-up What can be measured What outcomes can be achieved What to consider for data collection and reporting Cancer Surveillance prostate, breast, and head and neck cancers.34-39 Such findings have helped shed insight into the burden of cancer and differences in outcomes in comparison with US or global cancer statistics. Furthermore, the important role played by cancer registries, in terms of research or public health intervention, was illustrated by the French Caribbean island of Martinique.40-46 There, long-existing, precise registry data have informed analytical studies on several cancers and have helped assess the environmental and occupational health risks or evaluation of medical practice and local screening programs for major sites.40-46 Optimizing fruitful surveillance and research collaboration requires certain legal and ethical environments, as well as specific infrastructure and organization. Of particular importance is a properly defined and functioning information system. This should not • Legal/ethical issues • Infrastructure/ organization • Functioning information systems • Protocols and best practices • Feasibility and sustainability • Exhaustivity/quality of data coding/reporting • Rates and trends (incidence, mortality) • Clinical characteristics • Survival characteristics • Measuring impact/ outcomes of interventions introduced through research study C A N C E R C O N T R O L • Health care planning • Policy decision-making • Program planning and evaluation Fig 3. — Schematic of how surveillance data can be used to drive research studies through collaboration to promote cancer control. October 2015, Vol. 22, No. 4 Cancer Control 525 necessarily be electronic, but indicators must be well defined, with appropriate resources for sustaining data collection and reporting from both public and private health care sectors. In addition, standard protocols, and best practices, relying on evidence-based medicine, should be implemented. Several presenters during the workshop provided evidence for using cancer registries as building blocks for collaborative research, with particular emphasis on capacity building in developing regions. Surveillance data were also demonstrated to have use in driving research studies. For example, the purpose of any cancer registry should include producing more stable and accurate rates, greater access to data, and the possibility to investigate geographical variability (Fig 3). However, a registry would also be valuable to investigate the quality of care, possibly by looking at stage at diagnosis, or whether screening is being performed, as well as examinations of social issues and barriers to screening and treatment. Although separate research studies may be required for some of these, by working with registry teams, the data could be combined to provide a broader picture. Population-based cancer registries represent the gold standard, but they can also be used to examine rates standardized by age, age-specific incidence, sex ratios, rates in different ethnic groups and migrants, time trends, and survival.47-49 These data could then be used in health care planning and monitoring, as well as to inform policies, guide planning, and, once the knowledge of trends is available, to project treatment outcomes. When they are used to help validate current cancer mortality data, cancer registry data can also illuminate areas for research. Proving the usefulness of the data from a cancer registry is a powerful way to influence the public and to help ensure continued future funding. Linking surveillance with research outcomes is another way to guarantee continued collaboration and, perhaps, registry sustainability. For example, registry data can be used to evaluate improved outcomes from a range of research projects: public health interventions, comorbidity investigations, examination of the quality of diagnosis, treatment and follow-up care, epidemiological research, genetics, community impact, and survivorship, among others. Policy Decision-Making and Its Impact A general consensus suggested that the role of policy makers in institutions and governments is vital, because financial resources are essential to sustain a registry, especially in the current economic climate. This must be considered at a time when countries are grappling with salary payments and consideration of the sustainability of a registry may not be seen as a priority. Barbados represents an example of how surveillance might be made more cost effective.50,51 It has 526 Cancer Control a cancer and a multi–noncommunicable diseases registry, including data on stroke and acute myocardial infarction.50 Having 3 components to a registry can provide economies of scale, where separate registries may become too expensive. Shared leadership, office space, and training, combined with administrative, statistical, and epidemiological support across all registry components, can reduce costs. However, financial challenges remain because, as is similar across many countries, cancer registries operate without much publicity and the work they conduct may be largely unknown.24 Registry funds rarely include a line item for marketing or public advocacy, and, in many Caribbean countries, cancer is a taboo subject not openly discussed, so a registry rarely gains much public recognition and, hence, financial security for a cancer registry becomes even more precarious.24,51 A stepwise approach was proposed by the CARPHA to improve cancer registration in the region: strengthen existing registries and establish mechanisms to improve reporting (even if merely reporting on the type of data collected to date), perform situation analyses of what currently exists, outline a strategic plan in which strengths and weaknesses are identified, and assist with data analysis and reporting at least annually. Discussion A need exists in the Caribbean for a common, unified response and for more active regional collaboration in terms of cancer surveillance and research, which takes into account territorial specificities (similarities and differences) and implicates both scientific and governmental bodies. However, Caribbean countries vary in their urgency and capacity to respond to increasing regional rates of cancer burden.4,5,22,24 Sparse and incomplete data, aging populations, high-cancer risk prevalence, low/limited resource settings, as well as high frequencies of cancer-related deaths or cancers with poor prognoses, or the lack of targeted cancer care and prevention strategies, highlight the importance for progress in cancer surveillance and research.4,5,22 This is even more true when it comes to building synergy between these 2 fields for targeting efficient cancer control and management strategies in the Caribbean.4,5,22 Conscious of the need to move forward in the cancer field, different stakeholders in the region have joined their efforts to address challenges and opportunities for cancer surveillance and research in the Caribbean.4,5,22,24,25 Prior regional scientific meetings have helped provide better insight on the current state of surveillance infrastructure and collaborative research initiatives and outcomes.4,5,17,18,22,24,25 Although collaborations between research and surveillance have benefits and exist in the regional context, they are still mostly deemed insufficient.4,5,17,18,22,24,25 The state of colOctober 2015, Vol. 22, No. 4 laborative research between registries and medical/ research teams still remains limited and policymakers are not fully convinced of the need to promote such endeavors.27 Population-based cancer registries allow a specific type of surveillance vital for delivering quality national data to provide a precise description of the degree of cancer burden in a country. Without such data, knowledge about the degree of cancer burden may be very limited. Furthermore, it would be difficult, if not impossible, to develop, implement, monitor, and evaluate cancer strategies for prevention and disease management without cancer registries due to their continuous provision of national data.14,27-29 These data are important for cancer control and essential to better guide research.14,27-29 Most Caribbean nations with registries have managed to achieve an infrastructure that encourages collaboration with research; however, major challenges still exist to sustaining registry activity, addressing confidentiality concerns in a small island setting, maintaining economic viability and optimizing the quality of their data due to a combination of poor documentation and lack of electronic health information systems.22,24 For example, in Barbados, existing challenges include incomplete or poorly documented medical records, limitations in retrieval of death records, and suboptimal passive reporting from the private medical sector.22 One of the fundamentals will be stimulating better awareness of available tools and resources as well as resource optimization between existing Caribbean research networks, cancer registries, and international organizations. Other Caribbean countries, despite acknowledging their need, experience challenges from limited cancer registry expertise or infrastructure, and/or a lack of support from governmental bodies.22,24 Many of these countries have important cancer burdens despite their small geographical and population sizes.2,4,5,17,18,22,24 For example, in St Lucia, which has had a hospital-based cancer registry in operation since 1997, almost one-half of the cases registered in 2002 were identified from death certificates alone, and cancer has been the leading cause of death on the island since 2006.22 Fully registered cases often lack follow-up information, partly due to decentralization and duplication of patient records within the public health care sector and partly because of the number of patients sent overseas for treatment.22 In addition, despite being operational for more than 15 years, the St Lucian registry is not perceived as being indispensable.22 Implementation of a national cancer control program through a multisectoral approach, including more collaboration with international agencies and more experienced registries, may help address some of these issues. To help advance the cancer registry agenda, fledgling registries can take advantage of civil society orOctober 2015, Vol. 22, No. 4 ganizations. For example, in the Cayman Islands, the Cayman Islands Cancer Society has played a significant role in lobbying for the registry.22 In Barbados, local civil society organizations have been strong supporters of the national cancer registry, working in partnership with them to ensure comprehensive data capture.22,24 The potential role that civil society organizations can play in reaching communities to build awareness and create grass roots advocates for registries is important. They also provide a valuable link between potential community advocates and decision-makers in ministries of health. Further solutions lie in developing a strategic plan that includes setting standards for reinforced sustainable population research for the Caribbean. This implies identifying intercountry and intracountry specificities, needs, and priorities as well as existing tools, resources, and stakeholders. Developing standards also requires improved use of existing information systems, the generation of higher-quality cancer data, the implementation of best practice protocols, and more efficient communication. Because of the strong advocacy for surveillance guiding targeted research, the strategic regional research plan will also need to stimulate the development of collaborations in the region. Such collaborations, initiated between the various stakeholders/actors in the field of oncology (existing/aspiring cancer registries, research and medical teams, governmental and institutional policy makers and other cancer advocates/research teams at the local, regional and international levels), will create opportunities for dialogue on the rationale and role of cancer registration, the value, application, and utilization of cancer registry data in collaborative research, the illustration of the outcomes of cooperative work, and the importance and impact of policy decision-making. Capacity building will be imperative to facilitate research collaboration through linkages between cancer registries, clinical personnel, research teams, and diverse data sources (eg, histopathology, comorbidity), because even well-established cancer registries experience underutilization of their data as a research resource.22,24 For example, the French Caribbean islands of Martinique and Guadeloupe need to promote their scientific activities to develop collaboration between these French Caribbean registries and other Caribbean islands with populations of similar ethnic and environmental patterns.22,24 Another important issue for some islands is their small population sizes, which make it difficult to examine less common cancer sites.22 Collaborative studies involving several Caribbean nations can overcome this limitation; however, differences in social and cultural contexts, in economic development, and lack of data on ethnicity in the French registries may be significant barriers to implementation of such collaborative studies. Cancer Control 527 To overcome some of these barriers, the AC3 has integrated cancer registries (7 Caribbean and 2 African) as members of their network and has constituted a broad-based platform for education, training, and research on all cancer aspects (etiology, screening, prevention, treatment, and survival) in populations of African descent. During the 2012, 2013, and 2014 AC3 Caribbean meetings, the desire to see an increase in indigenous cancer statistics reports from the region was unanimous and regional analyses of cancer statistics were prioritized as one of its planned activities.5,22,25 Collaborative efforts between the cancer registries represented within the AC3 are currently in motion. At the forefront of collaborative research in the region with 45 published articles, the AC3 has several initiatives in progress for regional cancer data analyses, foundations for research biospecimen banks, networking, collaborating, standardizing, supporting, and optimizing existing cancer surveillance and research opportunities, with the main objective of establishing a global cancer network within the Caribbean region.21 The 2013 and 2014 AC3 cancer registration meetings in Barbados and Martinique provided a much-needed opportunity for networking between research investigators, cancer registrars, and health care professionals, providing insight into the possibility of collaborative opportunities between cancer registries within the wider Caribbean region.22,25 These meetings also emphasized the importance of a regional cancer network in the Caribbean to help promote collaborative research and to obtain more effective and efficient cancer control, as the Caribbean moves toward optimization of these registries for scientific excellence in cancer research.22,25 Existing research–cancer registry collaborations are paving the way, and organizations like the AC3, NCI, IARC, and PAHO facilitate and promote such endeavors.4,5,17,18,21,22,24,25 In 2011, heads of state and governmental representatives assembled for a high level UN meeting on the prevention and control of noncommunicable diseases.52 The meeting culminated with a political declaration, thus highlighting the need to reduce risk factors, strengthen national policies and health systems, build international cooperation, including collaborative partnerships, promote research and development, as well as implement systems for monitoring and evaluation to lessen the growing burden of noncommunicable diseases.52 Because cancer is a major noncommunicable disease that impacts morbidity and mortality in the Caribbean, cancer surveillance must be included as a key component in the global monitoring framework for noncommunicable disease prevention and control developed as a result of the political declaration of the United Nations on noncommunicable diseases. Therefore, Caribbean countries are obligated to collect 528 Cancer Control reliable data on cancer to facilitate reporting on these global indicators for noncommunicable diseases.22,24 Research on cancer is also limited in the Caribbean region and is greatly needed to facilitate a better understanding of this problem in the area, with a view to improving care and treatment as well as to guide the implementation of interventions for cancer prevention and control.4,5,17,18,21,22,24,25 The regional agency, the CARPHA, supports public health surveillance and research in the Englishand Dutch-speaking Caribbean.53 Working with its 23 member states, as well as with international partners, the agency is committed to strengthening the scientific evidence on cancers and other diseases of public health importance as a means of improving public health in the region.53 The NCI has made significant investments to international cancer research, training, and other programs, with the goal of reducing the global cancer burden.54 In 2011, these activities were formalized and coordinated via the creation of the Center for Global Health, which works to address the complex global challenges of cancer.54 One of the top priorities of the Center for Global Health is strengthening US national and international partnerships for collaborations in global health research, cancer research, prevention, and control relevant to developing countries.54 To pursue these goals, the NCI performed several site visits in the Caribbean with the aim of learning about the available infrastructure and priorities for cancer control planning.24 As a result, the NCI partnered with national, international, and regional stakeholders to plan initiatives to address the issue of cancer surveillance and registration for the Caribbean.24 The NCI is also a partner in the global initiative for developing cancer registries in low- and middle-income countries, which is led by the IARC and implemented through regional hubs that provide technical and scientific support, deliver tailored training for population-based cancer registration, advocate for cancer registration, affiliated associations and networks, and coordinate international research projects.24 Furthermore, the US NCI has partnered with the CARPHA, the PAHO, other US federal agencies as well as the AC3, to plan a stakeholder meeting for cancer surveillance to stimulate the strengthening of cancer registration and collaborative research in the Caribbean, to encourage the strategic use of information for national cancer control plans and programs, and to discuss strategies for moving forward.24 All of these activities will contribute to an overall strategic plan for the development and strengthening of cancer registration for the region. Conclusions Capacity building, resource optimization and sustainment, as well as collaboration and communication beOctober 2015, Vol. 22, No. 4 tween cancer surveillance and research teams, are key to obtaining robust and complete cancer surveillance data for the Caribbean region and advancing cancer control through collaborative research. The motto remains that we must collaborate to grow. Acknowledgments: The authors would like to thank the National Cancer Institute, Center for Global Health and Donald T. Simeon, MSc, PhD, Caribbean Public Health Agency, for support of the AC3 Cancer Registry Workshop during the CARPHA 2013 annual meeting. References 1. World Health Organization website. Cancer. http://www.who.int /cancer/en. Accessed November 11, 2015. 2. Ferlay J, Soerjomataram I, Ervik M, et al. eds. GLOBOCAN cancer incidence and mortality worldwide database. Vol 1. IARC CancerBase no. 11. 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Port-of-Spain, Trinidad and Tobago: WHO; January 2011. 32. Multigner L, Ndong JR, Giusti A, et al. Setting up of a health disparity index for the French West Indies and its application to the field of public health. Presented at: 3rd Inter-Regional Meeting of Health Surveillance in the French West Indies; Gosier, Guadeloupe; October 26–27, 2012. 33. Michel S, Banydeen R, Bakkhan-Mambir B, et al. Développement d’un indice de défavorisation pour les Antilles françaises et application en santé publique. Presented at: 3rd Journées interrégionales de Veille Sanitaire des Antilles Guyane; Gosier, Guadeloupe; October 26–27, 2012. 34. Guyana Cancer Registry Report 2000-2004. Ministry of Health Republic of Guyana; 2004. http://ghdx.healthdata.org/record/guyana -cancer-report-2000-2004. Accessed November 11, 2015. 35. Mutetwa B, Taioli E, Attong-Rogers A, et al. 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Evaluation of a cervical cancer screening campaign: reflections on the experience in Martinique [in French]. Sante Publique. 2000;12(spec no):21-35. 45.Dieye M, Quénel P, Goria S, et al. Study of the Geographical Distribution of Cancers According to Soil Contamination by Organochorine Pesticides in Martinique [in French]. Saint-Maurice, France; Institut de Veille Sanitaire; 2009. 46. Cardoso T, Dieye M, Alpha-Camy M. Investigation of Suspected Cancer Control 529 Cancer Clustering [in French]. Martinique, France: Cellule Interrégionale d’Epidémiologie; 2006. 47. Cook-Johnson D. A retrospective study (1976-1980) of the incidence and mortality of cancer of the breast, prostate, cervix, stomach and lung in Barbados (dissertation). University of the West Indies; Cave Hill Campus; 1986. 48. Curado MP, Edwards B, Shin HR, et al, eds. Cancer Incidence in Five Continents, Vol. IX. IARC scientific publication no. 160. Lyon, France: International Agency for Research on Cancer; 2007. 49. Mathers CD, Fat DM, Inoue M, et al. Counting the dead and what they died from: an assessment of the global status of cause of death data. Bull World Health Organ. 2005;83(3):171-177. 50. Barbados National Registry for Chronic Non-Communicable Disease. Barbados national registry. http://www.bnr.org.bb/cms. Accessed November 11, 2015. 51. Rose AMC, Hambleton IR, Jeyaseelan SM, et al. Establishing a national non-communicable disease surveillance system in a small-island state: Lessons learned from Barbados. Pan American J Public Health. [In press]. 52. World Health Organization. Resolution adopted by the General Assembly: political declaration of the high-level meeting of the General Assembly on the prevention and control of non-communicable diseases. New York: United Nations General Assembly; 2012. http://www.who.int /nmh/events/un_ncd_summit2011/political_declaration_en.pdf. Accessed November 11, 2015. 53. Caribbean Public Health Agency website. http://www.carpha.org. Accessed November 11, 2015. 54. National Cancer Institute Center for Global Health website. http:// www.cancer.gov/about-nci/organization/cgh. Accessed November 11, 2015. 530 Cancer Control October 2015, Vol. 22, No. 4 Ten Best Readings Relating to Palliative Care for Oncology Beller EM, van Driel ML, McGregor L, et al. Palliative pharmacological sedation for terminally ill adults. Cochrane Database Syst Rev. 2015;1:CD010206. Evidence relating to the effectiveness of palliative sedation in terms of symptom control or quality of life was insufficient. However, evidence suggests that death is not hastened by palliative sedation, but these data come from low-quality studies. Therefore, further studies are needed to specifically measure the effectiveness and quality of life in sedated people compared with nonsedated people as well as the potential for adverse events. Smith TJ, Temin S, Alesi ER, et al. American Society of Clinical Oncology provisional clinical opinion: the integration of palliative care into standard oncology care. J Clin Oncol. 2012;30(8):880-887. This provisional clinical opinion addresses the integration of palliative care services into standard oncology practices when a patient is diagnosed with advanced or metastatic cancer. Nielsen LM, Olesen AE, Branford R, et al. Association between human pain-related genotypes and variability in opioid analgesia: an updated review. Pain Pract. 2015;15(6):580-594. Studies have revealed promising results regarding use of pharmacogenetics as a diagnostic tool in the experimental setting; however, use of pharmacogenetics is a more complex task to accomplish in the clinical setting. Hui D, Bruera E. Models of integration of oncology and palliative care. Ann Palliat Med. 2015;4(3):89-98. The aim of this review was to study contemporary conceptual models and clinical approaches for integrating oncology with palliative care. The authors suggest that additional research is needed to advise best practices for integrating palliative care into various health care settings. Kamal AH, Gradison M, Maguire JM, et al. Quality measures for palliative care in patients with cancer: a systematic review. J Oncol Pract. 2014;10(4):281-287. These authors review a large group of measures used for assessing the quality of palliative care in patients with cancer and suggest that oncology-driven quality and outcomes studies are needed to broaden the connection between quality-based care and improved patient experiences. Pereira J, Green E, Molloy S, et al. Population-based standardized symptom screening: Cancer Care October 2015, Vol. 22, No. 4 Ontario’s Edmonton Symptom Assessment System and performance status initiatives. J Oncol Pract. 2014;10(3):212-214. Early evidence demonstrates the positive impact that population-based symptom screening has on patient care in Ontario, Canada. Expansion outside of hospitals and cancer centers accompanied by increased clinician engagement and education throughout the system is necessary to ensure the long-term success of the program. Hui D, Dos Santos R, Chisholm G, et al. Bedside clinical signs associated with impending death in patients with advanced cancer: preliminary findings of a prospective, longitudinal cohort study. Cancer. 2015;121(6):960-967. Five very specific physical signs associated with death within 3 days of onset in patients with cancer may aid in the diagnosis of impending death. In this study, the authors discuss the frequency and onset of 52 other bedside physical signs and examine their diagnostic performance. Bakitas MA, Tosteson TD, Li Z, et al. Early versus delayed initiation of concurrent palliative oncology care: patient outcomes in the ENABLE III randomized controlled trial. J Clin Oncol. 2015;33(13):1438-1445. Researchers investigated the effect of early compared with delayed palliative care on symptom impact, quality of life, mood, resource use, and 1-year survival rates. They suggest that understanding the complex mechanisms by which palliative care may improve survival rates remains an important research priority. Back AL, Park ER, Greer JA, et al. Clinician roles in early integrated palliative care for patients with advanced cancer: a qualitative study. J Palliat Med. 2014;17(11):1244-1248. Focus groups were conducted with palliative care clinicians who participated in randomized control trials of early palliative care for metastatic lung cancer. These data have become the foundation for training programs for clinicians. Levy MH, Smith T, Alvarez-Perez A, et al. Palliative care, version 1.2014. Featured updates to the NCCN guidelines. J Natl Compr Canc Netw. 2014;12(10):1379-1388. This report summarizes panel discussions from the National Comprehensive Cancer Network and updates to its guidelines from 2013 and 2014. The most updated version of these guidelines can be found at http://www. nccn.org/professionals/physician_gls/pdf/palliative.pdf. Cancer Control 531 Peer Reviewers, 2015 Peer review is the indispensable and critical element in the evaluation of all manuscripts being considered for publication in our journal. The oncology professionals who took part in our peer review process last year are listed below, and we thank them for their invaluable efforts on behalf of Cancer Control. — The Editor External Reviewers Agarwala, Sanjiv S, MD Temple University School of Medicine Alsirafy, Samy A, MBBCH, MSc, MD Cairo University, Egypt Andersen, Barbara L, PhD Ohio State University Asa, Sylvia, MD, PhD University of Toronto, Canada Bauman, Jessica R, MD Massachusetts General Hospital Cancer Center Bouvet, Michael, MD University of California San Diego Brody, Garry S, MD University of Southern California Bush, Shirley H, MBBS University of Ottawa, Ontario, Canada Chan, Jennifer A, MD University of Calgary, Canada Cui, Juwei, MD, PhD First Hospital of Jilin University, China Damato, Bertil E, MD, PhD University of California, San Francisco de Vries, Esther, PhD Erasmus University Medical Center, The Netherlands DeCoteau, John, MD University of Saskatchewan, Canada Greer, Joseph A, PhD Massachusetts General Hospital Cancer Center Gwak, Ho-Shin, MD, PhD National Cancer Center Korea Heegaard, Steffen, MD, DMSc University of Copenhagen, Denmark Henderson, Mark A, MD Oregon Health & Science University Vergo, Maxwell T, MD Dartmouth-Hitchcock Medical Center Köbel, Martin, MD University of Calgary, Alberta, Canada Wainstein, Alberto, MD, PhD Cirurgião Oncologico ONCAD, Brazil Lawlor, Peter G, MD University of Ottawa, Canada Watson, Peter H, MD BC Cancer Agency, Vancouver, British Columbia Ledezma, Rodrigo A, MD University of Chicago Medical Center Leland, June, MD James A. Haley VA Hospital Linos, Konstantinos, MD Dartmouth-Hitchcock Medical Center Lutgendorf, Susan K, PhD University of Iowa Mardini, Houssam, MD University of Kentucky Maryska Janssen-Heijnen, PhD VieCuri Medical Centre, The Netherlands Mehta, Vikas, MD Louisiana State University Health Shreveport Edwards, Brenda K, PhD National Cancer Institute Egloff, Ann Marie, MD University of Pittsburgh Fitzgibbons, Patrick L, MD St Jude Medical Center, California Fry, Lucia C, MD, PhD University of Alabama at Birmingham Gandhi, Manish J, MD Mayo Clinic, Rochester 532 Cancer Control Temple-Oberle, Claire, MD University of Calgary, Alberta, Canada Kim, Eric H, MD Washington University School of Medicine Done, Susan, PhD University of Toronto, Canada Dylewski, Mark, MD Baptist Health South Florida Stewart, Jonathan, MD James A. Haley VA Hospital Truini, Anna, PhD Istituto Nazionale per la Ricerca sul Cancro, Italy McMullen, Kevin P, MD Indiana University School of Medicine Duggan, Máire, MD University of Calgary, Alberta, Canada Roth, Patrick, MD University Hospital Zurich, Switzerland Hertz, Daniel L, PharmD, PhD University of Michigan College of Pharmacy Demetrick, Douglas J, PhD, MD University of Calgary, Canada Disclafani, Mark, MD Bradenton, Florida Park, John W, MD University of California, San Francisco Mehran, Resa, MD MD Anderson Cancer Center Meloni, Maria Franca, MD University of Milano-Bicocca, Italy Munkemuller, K, MD, PhD University of Alabama at Birmingham Nagahashi, Masayuki, MD Niigata University Graduate School of Medical and Dental Sciences, Japan Neumann, Helmut, MD University of Erlangen-Nuremberg, Germany Ozyigit, Gokhan, MD Hacettepe University, Ankara, Turkey Patel, Darpan, PhD University of Texas Health Science Center Patel, Jai N, PharmD Levine Cancer Institute Wiener, Lori, PhD National Institutes of Health Wood, Michael, MD University of North Carolina Zanagnolo, Vanna, MD European Institute of Oncology, Milan, Italy Zhang, David Y, MD, PhD Mount Sinai School of Medicine Internal Reviewers (H. Lee Moffitt Cancer Center & Research Institute) Almhanna, Khaldoun, MD Balducci, Lodovico, MD Benson, Kaaron, MD Chinnaiyan, Prakash, MD Choi, Junsung, MD Craig, David S, PharmD Dilling, Thomas J, MD Djulbegovic, Benjamin, MD Extermann, Martine, MD Gray, Jhanelle E, MD Hulse, Mark, RN Khoury, Michael, MD Loftus, Loretta, MD Magliocco, Anthony M, MD Morgan, Mary Ann, PhD, ARNP Portman, Diane, MD Pow-Sang, Julio M, MD Sahebjam, Solmaz, MD Shah, Bijal, MD Spiess, Phillip, MD Thirlwell, Sarah, MSc, MSc(A), RN Toloza, Eric M, MD Tran, Nam, MD October 2015, Vol. 22, No. 4 Index for 2015, Volume 22 Vol 22, No 1: Transfusion Medicine 4 Editorial: Transfusion Medicine Issues Pertaining to Patients With Cancer 102 Pathology Report: Familial Gastrointestinal Stromal Tumor Syndrome: Report of 2 Cases With KIT Exon 11 Mutation Derek H. Jones, MD, Jamie T. Caracciolo, MD, Pamela J. Hodul, MD, et al Kaaron Benson, MD 7 Safety of the Blood Supply German F. Leparc, MD 109 Tumor Biology: Current and Emerging Therapies for Bone Metastatic Castration-Resistant Prostate Cancer 16 Adverse Effects of Transfusion Jeremy S. Frieling, David Basanta, PhD, and Conor C. Lynch, PhD Radhika Dasararaju, MD, and Marisa B. Marques, MD 26 Clinical Effects of Red Blood Cell Storage Lirong Qu, MD, PhD, and Darrell J. Triulzi, MD 38 Transfusion Indications for Patients With Cancer Thomas Watkins, DO, PhD, Maria Katarzyna Vol 22, No 2: Molecular Biomarkers 133 Editorial: Personalizing the Attack on Cancer: Zeroing in on Targets Anthony M. Magliocco, MD Surowiecka, MD, and Jeffrey McCullough, MD 137 Practical Applications of Digital Pathology 47 Platelet Transfusion for Patients With Cancer Craig H. Fletcher, MD, Melkon G. DomBourian, MD, and Peter A. Millward, MD 52 Transfusion Support Issues in Hematopoietic Stem Cell Transplantation Claudia S. Cohn, MD, PhD 60 Therapeutic Apheresis for Patients With Cancer Laura S. Connelly-Smith, MBBCh, DM, and Michael L. Linenberger, MD 79 Using HLA Typing to Support Patients With Cancer Daryoush Saeed-Vafa, MD, and Anthony M. Magliocco, MD 142 Molecular Technologies in the Clinical Diagnostic Laboratory Victor E. Zota, MD, and Anthony M. Magliocco, MD 152 Molecular Assays in Cytopathology for Thyroid Cancer Pablo Valderrabano, MD, Victor E. Zota, MD, Bryan McIver, MD, PhD, et al 158 Biomarkers in Hematological Malignancies: A Review of Molecular Testing in Hematopathology Mohammad Hussaini, MD Mark K. Fung, MD, PhD, and Kaaron Benson, MD 87 Special Report: Mobilization and Transplantation Patterns of Autologous Hematopoietic Stem Cells in Multiple Myeloma and Non-Hodgkin Lymphoma Luciano J. Costa, MD, PhD, Shaji Kumar, MD, Stephanie A. Stowell, MPhil, et al 95 Special Report: Functional Health Literacy, Chemotherapy Decisions, and Outcomes Among a Colorectal Cancer Cohort Evan L. Busch, MPH, Christopher Martin, MSPH, Darren A. DeWalt, MD, et al October 2015, Vol. 22, No. 4 167 Circulating Tumor Cells: A Window Into Tumor Development and Therapeutic Effectiveness Gisela Cáceres, PhD, John A. Puskas, PhD, and Anthony M. Magliocco, MD 177 Surgical and Molecular Pathology of Barrett Esophagus Sherma Zibadi, MD, PhD, and Domenico Coppola, MD 186 Molecular Pathology of Soft-Tissue Neoplasms and Its Role in Clinical Practice Evita B. Henderson-Jackson, MD, and Marilyn M. Bui, MD, PhD Cancer Control 533 Index for 2015, Volume 22 193 Advances in EGFR as a Predictive Marker in Lung Adenocarcinoma Farah K. Khalil, MD, and Soner Altiok, MD, PhD 261 Editorial: Does Androgen Deprivation Therapy for Prostate Cancer Increase the Risk of Colorectal Cancer? Anna Martling, MD, PhD 200 Impending Impact of Molecular Pathology on Classifying Adult Diffuse Gliomas Robert J. Macaulay, MD 206 Overexpression of Vascular Endothelial Growth Factor A in Invasive Micropapillary Colorectal Carcinoma Marilin Rosa, MD, Maisoun Abdelbaqi, MD, Katherine M. Bui, et al 211 Advances in the Molecular Analysis of Breast Cancer: Pathway Toward Personalized Medicine Marilin Rosa, MD 220 Use of Immunohistochemical Stains in Epithelial Lesions of the Breast Laila Khazai, MD, and Marilin Rosa, MD 226 Special Technologies for Ex Vivo Analysis of Cancer Jenny M. Kreahling, PhD, and Soner Altiok, MD, PhD 232 Editorial: Should We Preclude Phase 1 Clinical Trials From Enrolling Elderly Individuals? Lodovico Balducci, MD 235 Original Research: Effect of Age on Clinical Outcomes in Phase 1 Trial Participants Amit Mahipal, MD, Aaron C. Denson, MD, Benjamin Djulbegovic, MD, PhD, et al 242 Case Report: Primary Enteropathy-Associated T-Cell Lymphoma Type 2: An Emerging Entity? Nicole M. Grigg-Gutierrez, MD, Rodolfo Estremera- Marcial, MD, William W. Cáceres, MD, et al 248 Special Report: Cost Effectiveness of Colorectal Cancer Screening Strategies Shaan S. Patel and Meredith L. Kilgore, PhD 259 Editorial: Colorectal Cancer: The Last Nail in the Coffin for Androgen Deprivation Therapy for Prostate Cancer? Lodovico Balducci, MD, and Julio M. Pow-Sang, MD 534 Cancer Control 263 Special Report: Risk of Colorectal Cancer by Subsite in a Swedish Prostate Cancer Cohort Yunxia Lu, MD, PhD, Rickard Ljung, MD, PhD, Anna Martling, MD, PhD, et al Vol 22, No 3: Robotic-Assisted Surgery 278 Editorial: Applications and Advances in Robotic-Assisted Oncological Surgery: Ready to Dock the ‘Bot Eric M. Toloza, MD, PhD, and Julio M. Pow-Sang, MD 283 Robotic-Assisted Laparoscopic Radical Prostatectomy Gautum Agarwal, MD, Oscar Valderrama, MD, Adam M. Luchey, MD, et al 291 Robotic-Assisted Renal Surgery Justin B. Emtage, MD, Gautum Agarwal, MD, and Wade J. Sexton, MD 301 Robotic-Assisted Radical Cystectomy Adam M. Luchey, MD, Gautum Agarwal, MD, and Michael A. Poch, MD 307 Robotic-Assisted Surgery in Gynecological Oncology Stephen H. Bush, MD, and Sachin M. Apte, MD 314 Robotic-Assisted Videothoracoscopic Surgery of the Lung Frank O. Velez-Cubian, MD, Emily P. Ng, Jacques P. Fontaine, MD, et al 326 Robotic-Assisted Videothoracoscopic Mediastinal Surgery David M. Straughan, MD, Jacques P. Fontaine, MD, and Eric M. Toloza, MD, PhD 331 Robotic-Assisted Surgery in the Head and Neck Jon Burton, MD, Robert Wong, MD, and Tapan Padhya, MD October 2015, Vol. 22, No. 4 Index for 2015, Volume 22 335 Robotic-Assisted Esophageal Surgery David M. Straughan, MD, Saїd C. Azoury, MD, Robert D. Bennett, MD, et al 403 Palliative Pharmacotherapy: State-of-the-Art Management of Symptoms in Patients With Cancer Eric E. Prommer, MD 340 Robotic Whipple Procedure for Pancreatic Cancer: The Moffitt Cancer Center Pathway Omar M. Rashid, MD, JD, John E. Mullinax, MD, Jose M. Pimiento, MD, et al 412 Pharmacological Management of Cancer Related Pain Eric E. Prommer, MD 352 Robotics in Neurosurgery: Evolution, Current Challenges, and Compromises 426 Clinical Implications of Opioid Pharmacogenomics in Patients With Cancer James J. Doulgeris, MSME, Sabrina A. Gonzalez- Blohm, MSBE, Andreas K. Filis, MD, et al Gillian C. Bell, PharmD, Kristine A. Donovan, PhD, and Howard L. McLeod, PharmD 360 Special Report: Association of Lymphoma- genesis and the Reactivation of Hepatitis B Virus in Non-Hodgkin Lymphoma 433 Palliative Sedation in Patients With Cancer Samir Dalia, MD, Yaman Suleiman, MD, David W. Croy, MD, et al 442 Integrating Psychosocial Care Into Routine Cancer Care Marco Maltoni, MD, and Elisabetta Setola, MD Paul B. Jacobsen, PhD, and Morgan Lee, MA 366 Case Report: Stromal Overgrowth in a Brenner Tumor or Ovarian Fibroma With Minor Sex Cord Elements? Julia A. Ross, MD, PhD, and Ozlen Saglam, MD 369 Case Report: Recurrent Systemic Anaplastic Lymphoma Kinase–Negative Anaplastic Large Cell Lymphoma Presenting as a Breast Implant–Associated Lesion Amanda Zimmerman, MD, Frederick L. Locke, MD, Josephine Emole, MD, et al 450 Systematic Review of Palliative Care in the Rural Setting Marie A. Bakitas, DNSc, CRNP, Ronit Elk, PhD, Meka Astin, MPH, et al 465 New Frontiers in Outpatient Palliative Care for Patients With Cancer Michael W. Rabow, MD, Constance Dahlin, ANP-BC, ACHPN, Brook Calton, MD, et al 475 Palliative Care in Adolescents and Young Adults With Cancer Vol 22, No 4: Perspectives, Progress, and Opportunities for Palliative Care in Oncology Kristine A. Donovan, PhD, Dianne Knight, MD, and Gwendolyn P. Quinn, PhD 480 Palliative Care in Older Patients With Cancer 382 Editorial: Perspectives, Progress, and Opportunities for Palliative Care in Oncology Lodovico Balducci, MD, Dawn Dolan, PharmD, and Sarah A. Hoffe, MD Diane Portman, MD, and Sarah Thirlwell, MSc, MSc(A), RN 386 Integrating Palliative Care Into Oncology: A Way Forward Kavitha Ramchandran, MD, Erika Tribett, MPH, Brian Dietrich, MD, et al 396 Improving the Quality of Palliative Care Through National and Regional Collaboration Efforts Arif H. Kamal, MD, MHS, Krista L. Harrison, PhD, Marie Bakitas, DNSc, CRNP, et al October 2015, Vol. 22, No. 4 489 Prognostication of Survival in Patients With Advanced Cancer: Predicting the Unpredictable? David Hui, MD, MSc 498 Pathology Perspective: Is DOG1 Immuno- reactivity Specific to Gastrointestinal Stromal Tumor? William Swalchick, MA, Rania Shamekh, MD, and Marilyn M. Bui, MD, PhD Cancer Control 535 Index for 2015, Volume 22 505 Case Report: Occurrence of Multiple Tumors in a Patient Elaine Tan, Mark Friedman, MD, and Domenico Coppola, MD 508 Special Report: Association of Cancer Stem Cell Markers With Aggressive Tumor Features in Papillary Thyroid Carcinoma Zhenzhen Lin, MD, Xuemian Lu, MD, Weihua Li, MD, et al 515 Special Report: Should Vital Signs Be Routinely Obtained Prior to Intravenous Chemotherapy? Results From a 2-Center Study Smitha Menon, MD, Nathan R. Foster, MS, Sherry Looker, RN, BSN, et al 520 Special Report: Advancing Cancer Control Through Research and Cancer Registry Collaborations in the Caribbean Rishika Banydeen, MPH, Angela M.C. Rose, MSc, Damali Martin, PhD, MPH, et al Vol 22, No 4: Supplement 2 Why a Special Issue on Chronic Lymphocytic Leukemia Lodovico Balducci, MD 3 Chronic Lymphocytic Leukemia in the Elderly: Epidemiology and Proposed Patient-Related Approach Lodovico Balducci, MD, and Dawn Dolan, PharmD 7 Chronic Lymphocytic Leukemia in the Elderly, Which Investigations Are Necessary: A Map for the Practicing Oncologist Javier Pinilla-Ibarz, MD, PhD, and Josephine Emole, MD 17 Management of Chronic Lymphocytic Leukemia in the Elderly Jacqueline C. Barrientos, MD 536 Cancer Control October 2015, Vol. 22, No. 4 Current Perspectives in Oncology Nursing February 18-19, 2016 Moffitt Cancer Center Tampa, Florida 15th Annual Nursing Conference SPECIAL EVENT The 21st Annual Rhinehart Reception & Lecture February 17, 2016 Moffitt Cancer Center Vincent Stabile Research Building 12902 Magnolia Drive Tampa, FL 33612 For More Information & Registration Visit our website at www.MOFFITT.org/Nursing2016 10th Annual Business of Biotech Conference Friday, March 18, 2016 7:45 a.m. – 3:00 p.m. Vincent A. Stabile Research Building 12902 Magnolia Drive, Tampa, Florida 33612 To register visit www.moffittip.com/events SCIENCE. SYNERGY. sUCCESS! Keynote - Arie Belldegrun, M.D. Sam Donaldson President, CEO and founder of Kite Pharma with a distinguished tenure in the life sciences field and closely involved with the founding and advancement of successful biopharmaceutical companies. Former ABC news correspondent and member of the Moffitt Board of Advisors conducts a conversation with Dr. Belldegrun about the exciting immunotherapy field for cancer treatment. Business of #MoffittBOB Biotech Moffitt Cancer Center