Pediatric Oncology for the
Primary Care Provider
Kate A. Mazur, MSN, RN, CPNP
Texas Children’s Hospital Advanced Practice Provider
1st Annual Conference
Houston, TX
February 8, 2014
Objectives
• Review the incidence of childhood cancer
• Describe the most common childhood
malignancies, with a focus on initial evaluation
and diagnosis, red flags, and when to refer to
specialist
• Analyze the unique precautions for patients with
oncologic illnesses
The Scope of the Problem
• 13,400 children ages birth to 19 years are
diagnosed with cancer each year
• Cancer is the #1 cause of disease-related death
in children
Source: Surveillance, Epidemiology, and End Results Program,
1975-2003, Div. of Cancer Control and Pop. Sciences, NCI, 2006
The Good News…
• Overall 5 year survival rate across all cancers is
now 80%
• Estimated 270,000 childhood cancer survivors
in the U.S.
• However, two-thirds of survivors face at least
one chronic health problem
• 25% of survivors face a late effect from
treatment that is classified as severe or lifethreatening
Where do I start?
• Early diagnosis and initiation of treatment is imperative
to improved survival
• Diagnosis of cancer starts with a thorough history and
physical
• Most symptoms of childhood cancer due to either a
mass, its effect on the surrounding tissues, invasion of
the bone marrow, or secretion of a substance by the
tumor that disturbs normal functions
• Most common presenting signs and symptoms of many
malignancies include weight loss, failure to thrive,
anorexia, malaise, fever, pallor, and lymphadenopathy
High Risk Patients
• Familial cancer pre-disposition syndromes
– Li-Fraumeni Syndrome
– Familial adenomatosis polyposis
– Beckwith-Wiedemann Syndrome
• Family history of cancer
– Retinoblastoma
• Autoimmune diseases in patient or family
• Down Syndrome
• Neurofibromatosis Type 1
• HIV/AIDS
Leukemia
•
Cancer of the bone marrow
•
Uncontrolled proliferation of immature white blood cells (“blasts”)
•
Most common type of cancer in children and adolescents under 20 years of age
(~30%)
•
Incidence
•
–
4900 new cases each year
–
Males > Females; Hispanics > Caucasians > African Americans
–
Peak age 2-5 years
Risk factors
–
Down Syndrome: 14-fold increase
–
Klinefelter Syndrome
–
Fanconi anemia
–
Immunodeficiencies: ataxia telangiectasia, Wiskott-Aldrich, Bloom syndrome
–
Past exposure to chemotherapy or ionizing radiation
Most Common Types of
Childhood Leukemia
• Acute lymphoblastic leukemia (ALL) ~ 80%
• Acute myelogenous leukemia (AML) ~ 15%
• Chronic myelogenous leukemia (CML) ~ 5%
Leukemia: Presenting Signs and
Symptoms
• Relate to the infiltration of the bone marrow
• History
– Fatigue
– Persistent fevers
– Bone pain
– Anorexia
– Weight loss
– Recurrent infections
• Physical Exam
– Pallor
– Bleeding, bruising, or petechiae
– Hepatosplenomegaly
– Lymphadenopathy
Lymphadenopathy: When to Worry
• Supraclavicular, axillary, or epitrochlear adenopathy
• Adenopathy that persists longer than 6 weeks or is increasing in size
• Asymmetric lymphadenopathy
• Leukemia usually presents with generalized lymphadenopathy;
localized LAD more likely to be infectious in origin
• Malignant nodes generally hard and nontender;
infectious/inflammatory nodes usually tender, freely moveable,
overlying erythema
• For localized cervical adenitis with inflammation and fever, treat with
ONE course of antibiotics, if no response  refer
• DO NOT START STEROIDS
Leukemia: Initial Diagnostic Tests
• CBC with manual differential, reticulocyte count, and
peripheral blood smear
• Chem 10: Electrolytes, BUN, creatinine, Ca2+, Mg2+,
PO4-, uric acid, LDH
– Hypocalcemia, hyperkalemia, hyperphosphatemia,
hyperuricemia may indicate tumor lysis syndrome 
oncologic emergency
– Elevated LDH (non-specific tumor marker)
• Bone marrow aspirate and biopsy is the only
definitive diagnostic test
– Done by oncology service only
Complete Blood Count: A Review
• White blood cells/Neutrophils
– Responsible for fighting
infection
• Red blood cells/Hemoglobin
– Carries O2 from lungs to
blood tissues and CO2 from
tissue to lungs
• Platelets
– Necessary for clotting and
control of bleeding
CBC Findings in Leukemia
• Leukocytosis - ~50% with WBC >10,000 and
~20% > 50,000
– Elevated blasts and low neutrophils
• Anemia
• Thrombocytopenia
• 2 or more cell lines decreased  REFER
– 90% present with hemoglobin < 11g/dL, 45% < 7g/dL
– 75% present with platelets < 100,000/mm3
Lymphoma
• Tumor of the lymphatic system
• Incidence
– 1500 new cases each year
– More common in adolescents and older teenagers, males, Caucasians
• Risk factors
– Immunodeficiency syndromes: Wiskott Aldrich, SCIDS, HIV/AIDS
– Autoimmune diseases: RA, SLE
– Some viruses: Epstein-Barr (particularly for Burkitt’s lymphoma)
Types of Lymphoma
• Non-Hodgkin’s Lymphoma – 60%
–
–
–
–
Lymphoblastic
Anaplastic
Burkitt’s
Diffuse large B-cell
• Hodgkin’s Lymphoma – 40%
Lymphoma: Presenting Signs and
Symptoms
• History – 75% asymptomatic
– Unexplained fever for more than 3 days
– Weight loss of 10% within 6 months
– Drenching night sweats
– Malaise
– Anorexia
• Physical Exam – based on location of disease
– Head/Neck: supraclavicular or cervical adenopathy, jaw
swelling, unilateral tonsillar enlargement
– Abdomen: splenomegaly, abdominal distention, jaundice
– Mediastinum: cough, chest pain, stridor
Lymphoma: Evaluation and Diagnostic
Work Up
• DO NOT START STEROIDS
• Blood work: CBC w/diff, liver and renal fxn tests, including alkaline
phosphatase; ESR, ferritin, LDH may be elevated
• Imaging Studies
– CT scans (neck, chest, abdomen, pelvis)
– PET scan
– Bone scan
• Biopsy of affected node
– Required for definitive diagnosis
– Done under guidance of oncology team only
Brain Tumors
• Can be malignant or “benign”
• Much slower rate of treatment advances than other malignancies
• Unique challenges in children – brain still developing
• Incidence
– 3400 new cases every year
– More common in ages < 15 years
• Risk factors
– Little known
– Hereditary cancer predisposition syndromes (i.e. Li-Fraumeni Syndrome)
Most Common CNS Tumors
• Astrocytoma
– Most supratentorial
but can originate in
cerebellum, brainstem,
or hypothalamus
• Brain Stem Glioma
– Medulla, pons
• Medulloblastoma
– Cerebellum
• Ependymoma
Picture from The Children’s Hospital at Montefiore,
http://www.montekids.org/services/neurosurgery/neurologicaldisorders/brain_tu
mor/
– Ependymal tissue within the ventricular system,
most commonly the fourth ventricle
CNS Tumors: Presenting Signs and
Symptoms
•
Depends on site and severity of disease as well as child’s age & development
•
History
– Seizures
– Visual changes
– Headache
– Nausea/vomiting, often in morning
– Poor concentration or mental status change
•
Physical Exam
– Hemiparesis
– Endocrinopathies
– Ataxia
– Cranial nerve deficits
Headache: When Further Evaluation is
Warranted
•
•
•
•
•
•
•
•
•
Recurrent morning headache
Headache that awakens the child
Intense, incapacitating headache
Changes in the quality, frequency, or pattern of
headaches
Presence or onset of neurologic abnormality
Ocular findings such as papilledema, decreased visual
acuity, or loss of vision
Associated with vomiting that is persistent, increasing in
frequency, or preceded by recurrent headaches
Age 3 years or less
DO NOT START STEROIDS
CNS Tumors: Diagnostic Tests
• Imaging studies are the only
diagnostic tests
• MRI brain and spine
• CT often obtained first due to
easy access
• LP if clinically
indicated and safe
to perform
Solid Tumors: Evaluation of an
Abdominal Mass
•
ALWAYS suspect a malignant solid tumor in a child with a palpable
abdominal mass
•
Avoid abdominal palpation as much as possible; palpate gently if necessary
•
In younger children, often renal  neuroblastoma or Wilms’ tumor
•
In older patients, may be related to leukemia or lymphoma with enlargement
of the spleen or liver
•
Obtain comprehensive GU and GI histories
•
Associated symptoms of flushing, palpitations, diarrhea, failure to thrive, and
fever could point to disseminated process such as neuroblastoma
•
Complete exam with focus on skin, extremities (bone pain), neuro exam
(Horner’s syndrome, spinal cord compression), organomegaly, measurement
of abdominal girth
•
Obtain diagnostic imaging STAT
– Usually start with abdominal ultrasound
Wilm’s Tumor
•
Large, rapidly growing, vascular renal tumor
•
Second most common intra-abdominal malignancy in children
•
Can be bilateral or unilateral
•
Incidence
•
–
500 new cases/year
–
Peak incidence 3 years of age
–
Slight female predominance; African-Americans > Caucasians > Asians
Risk factors
–
Congenital anomalies: GU malformations, hemihypertrophy, Beckwith-Wiedemann syndrome,
Denys-Drash syndrome
–
Familial pre-disposition syndromes: Li-Fraumeni syndrome
Wilm’s Tumor: History and PE Findings
• Abdominal mass – smooth, firm, rarely crosses midline but 510% are bilateral
• Diffuse abdominal distention with large masses
• Usually asymptomatic, but 25% have abdominal pain,
vomiting, hematuria, hypertension
• Signs of thrombosis: leg swelling, prominent veins over
abdomen
• Signs of hemorrhage into tumor (occurs rarely): anemia, fever,
rapid abdominal distension
Wilm’s Tumor: Diagnostic Tests
• Blood work – CBC, liver function, renal function
• Coagulation screen – may acquire Von Willebrand’s
• Abdominal ultrasound usually first test ordered – will
reveal mass arising from within kidney
• Doppler US to assess patency of renal vein and inferior
vena cava
• Abdominal CT
Neuroblastoma
•
Cancer of the sympathetic nervous system; derived from neural crest cells
•
Second most common solid tumor of childhood and the most common
extracranial solid tumor
•
Incidence
•
–
650 new cases every year in the U.S.
–
90% cases in children < 5 years old
–
Boys > girls; Caucasian predominance
Risk factors
– Most cases sporadic
– Has been identified in other disorders of neural crest cells
• Neurofibromatosis
• Hirschsprung’s disease
• Beckwith-Wiedemann syndrome
• DiGeorge syndrome
Neuroblastoma: Clinical Presentation
• Can arise anywhere along sympathetic nervous system
• Signs and symptoms depend on location of primary tumor and
presence of metastasis
• Two-thirds have primary site in abdomen, usually adrenal
• Thoracic region is the next most common primary site
• About 60% have metastatic disease at presentation due to vague
initial symptoms and late presentation
– Bone marrow
– Bone
– Liver
– Skin
– Orbits
Neuroblastoma: Presenting Signs and
Symptoms
•
•
•
•
•
•
•
•
•
•
•
Firm, irregular, non-tender abdominal mass
Hepatomegaly
Abdominal pain
Urinary obstructions
Flushing
Sweating
Diarrhea
Anorexia
Malaise
Site-specific symptoms from metastases to bone, skin, liver, or CNS
Opsoclonus-myoclonus syndrome – “dancing eyes and dancing
feet”
Neuroblastoma: Presenting Signs and
Symptoms
Neuroblastoma: Diagnostic Tests
•
•
•
•
•
•
•
•
CBC – cytopenias
Chem 10
Ferritin, LDH, uric acid
Liver panel
Urine catecholamines (VMA/HVA)
CT chest/abdomen/pelvis
Tissue biopsy for definitive diagnosis
Bilateral bone marrow aspirate and biopsy to evaluate
for BM involvement
• MIBG scan or bone scan to evaluate for metastasis
MIBG Scan
Osteosarcoma
•
Tumor of the bone; usually at the end of long bones
•
Incidence
– 400 new cases/year
– More prominent during adolescent growth spurt; periods of rapid bone growth
– Males > females; African-Americans > Caucasians
•
Risk factors
– Ionizing radiation
– Hereditary retinoblastoma
– Li-Fraumeni Syndrome
Osteosarcoma: Signs and Symptoms
•
•
•
•
•
Dull, aching pain, usually worse at night
+/- soft tissue swelling, warmth
May have vascularity over the mass
Decreased range of motion
Often long duration of symptoms prior to
diagnosis; can be up to 6 months
Osteosarcoma: Diagnostic Tests
• Diagnostic Imaging
– Plain radiograph of affected area – “sunburst
pattern”
– MRI to further examine tumor boundaries, soft
tissue component, relationship to joints, blood
vessels, neurovascular bundle
– Chest XR or CT to evaluate for mets
– Bone scan to evaluate for skeletal mets
• Blood Tests
– Elevations in LDH, alk phos may be present
• Tumor Biopsy
– Necessary for definitive diagnosis
Supportive Care of a Patient with Cancer
Infection Prophylaxis
• Good hand washing!!
• Proper dental hygiene – daily brushing with a soft brush, use of
chlorhexidine mouth rinse
• Avoidance of crowded, enclosed spaces
• Cleanliness of perirectal area – avoid constipation
• No rectal temperatures!
• Prophylaxis for Pneumocystis carinii pneumonia (PCP) from time of
diagnosis until 6 months after completion of therapy – Trimethoprimsulfamethoxazole, Pentamidine, Dapsone
• Viral and fungal prophylaxis may be indicated
Management of Fever in the Child with
Cancer
• Defined as a single temperature of ≥ 101°F or two temperatures ≥
100.4°F taken 1 hour apart
• Do not give anti-pyretics!
• No rectal temperatures
• Every cancer patient who presents with fever should be considered
septic until proven otherwise
• Prompt evaluation and management essential for improved survival
• Goal is to administer first dose of IV antibiotics within 1 hour of
presentation
Initial Evaluation of Fever
• Prompt tx essential – send to ER or urgent care center for immediate
evaluation and management and call oncology service
• Evaluate for signs of septic shock
– Tachycardia – usually the first sign of shock. Ideally, aggressive treatment starts
here.
– Check perfusion – delayed capillary refill; pulses weak and thready or bounding
– Hypotension – LATE sign
– Mental status changes; lethargy – OMINOUS
• Examine for focal signs of infection
– Often there are none
– Oral cavity, perianal area, skin, respiratory tract, abdomen (typhlitis)
Fever: Diagnostic Work Up
•
CBC with differential – risk-based management based on neutrophil count
•
Blood cultures – all CVC lumens and peripheral; important to have prior to
first dose of antibiotics
•
Urinalysis/urine culture, if feasible based on clinical status – no
catheterization
•
Additional cultures of any potential sources of infection – skin lesions, stool
sample if diarrhea, mouth sores
•
Do NOT I & D any skin lesions
•
Chest XR if respiratory symptoms – obtain portable or delay until after initial
antibiotics
•
Do NOT perform LP until oncology service is consulted
Empiric Therapy of the Febrile Patient
• DO NOT wait for culture results
• Broad-spectrum antibiotics must be started immediately; will tailor
antibiotic choice when culture results known
• Antibiotics chosen based on individual institution’s local microbial
prevalence and antibiotic susceptibility patterns
• Regimen also based on risk criteria – high risk if ANC < 100, infant
ALL, AML, in any phase of leukemia tx other than maintenance
(induction at highest risk), < 7 days from last chemotherapy, focal
signs of infection, or concern for sepsis
• Admission often indicated
• Anti-virals and/or anti-fungals may be added as clinically indicated
• Septic shock treated aggressively with normal saline
fluid boluses and triple antibiotics
Immunizations and the Cancer Patient
• Cannot assume adequate immunization even if patient’s vaccines
are up to date prior to diagnosis
• NO LIVE VACCINES
• Siblings or household contacts should not receive the oral polio
vaccine
• All other vaccines may continue as scheduled, but will need to check
titers at completion of treatment to ensure adequate immunity.
Boosters or full re-immunization may be needed after completion of
therapy
• Highly recommend seasonal influenza vaccination in patient and
household contacts
References
•
Howlader N. et al. SEER Cancer Statistics Review, 1975-2010, National
Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2010/,
based on November 2012 SEER data submission, posted to the SEER web
site, April 2013.
•
Hastings, C. (2002). The Children’s Hospital Oakland Hematology/Oncology
Handbook. St. Louis, MO: Mosby, Inc.
•
Kline, N. & Tomlinson, D. (2005). Pediatric Oncology Nursing. Heidelberg,
Germany: Springer Publishing.
•
Lowry, A., Bhakta, K., & Nag, P. (2011). Texas Children’s Hospital
Handbook of Pediatrics and Neonatology. McGraw-Hill Publishing.
•
Pizzo, P. & Poplack, D. (2005). Principles and Practice of Pediatric
Oncology, 5th edition. Philadelphia, PA: Lippincott, Williams & Wilkins.
•
Zorc, J. (2013). Clinical Handbook of Pediatrics, 5th edition. Philadelphia, PA:
Lippincott, Williams & Wilkins.
Questions??