John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Atlas of Radiological Modalities in the Evaluation of Ampullary Masses John B. Moore, MSc, HMSIII Gillian Lieberman, MD John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Agenda • • • • • • • • Patient Presentation Normal anatomy of the hepato-pancreatico-biliary system Differential diagnosis for a periampullary mass Menu of tests Radiological evaluation of the ampulla of Vater Categorizing the lesion with imaging Discussion of ampullary carcinoma Correlating findings with prognosis 2 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: HPI • 58 yo M who presented with symptomatic obstructive jaundice. Developed pruritus and dark urine of 3 weeks duration before presentation. Mild weight loss over past 3 months. No abdominal pain, nausea, vomiting, alcohol use. – PMH significant only for glaucoma – takes timolol – Father passed away from unknown GI malignancy • Labs were significant for following: – – – – – ALT: 192 IU/L AST: 133 IU/L Total bilirubin: 1.9 mg/dl Lipase: 1498 IU/L CA 19-9: 36 (normal < 34 U/ml) 3 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: Initial CT+ contrast CBD Pancreatic duct Pancreas Splenic artery Celiac artery SMV IVC Aorta C+ axial CT abdomen & pelvis BIDMC, PACS 4 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: Initial CT+ contrast The common bile duct and the pancreatic duct are dilated. This finding is known as the “double duct sign” C+ axial CT abdomen & pelvis BIDMC, PACS 5 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: Initial CT+ contrast C+ coronal CT abdomen & pelvis BIDMC, PACS Dilated common bile duct and pancreatic duct on coronal imaging C+ coronal CT abdomen & pelvis BIDMC, PACS Distended gallbladder in absence of clear obstructing lesions or stones 6 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: Initial CT+ contrast C+ coronal CT abdomen & pelvis BIDMC, PACS C+ coronal CT abdomen & pelvis Duodenum with pancreatic duct and CBD converging in periampullary region. BIDMC, PACS No overt mass seen 7 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Anatomy of the periampullary region 8 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Anatomy of the ampulla From Martin & Moser, Ampullary carcinoma, UpToDate 9 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Differential Diagnosis • Benign periampullary masses – Duodenal adenoma – Ampullary adenoma – Gallstones (choledocholithiasis or gallstone pancreatitis) • Periampullary cancers – Pancreatic ductal adenocarcinoma – Carcinoma of the bile duct • Cholangiocarcinoma (extra-hepatic) – Carcinoma of the ampulla itself – Carcinoma of the periampullary duodenum 10 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Let’s continue with several CT images of companion patients with other periampullary cancers. 11 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Companion Patient 2: Cholangiocarcinoma on CT C+ axial CT abdomen & pelvis BIDMC, PACS Ill-defined hypodense mass seen near common bile duct stent with a dilated gastroduodenal artery. The pancreas is atrophic. 12 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Companion Patient 3: Pancreatic adenocarcinoma on CT C+ axial CT abdomen & pelvis BIDMC, PACS Hypoenhancing mass in pancreatic head consistent with pancreatic adenocarcinoma. 13 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Menu of tests • • • • • • • Transabdominal US Abdominal CT MR and MRCP ERCP EUS IDUS Percutaneous transhepatic cholangiography (PTC) 14 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Transabdominal US • First imaging technique in pts with jaundice • Can potentially assess vascular involvement, biliary dilation, liver lesions • Overall accuracy in finding ampullary masses only 15% – If no gallstones or obvious pancreatic head mass → proceed to other modality • For patient 1, US was not initially done – No abdominal pain, low suspicion for stones 15 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 CT – abdominal • Once ampullary mass suspected → order “pancreatic mass protocol” • Water as “oral contrast” and IV contrast – water distends duodenum but w/o high attenuation of usual contrast • allows vessels to be clearly visualized – contrast allows for arterial- and venous-phase imaging • Acquire images 1.0 to 2.5 mm intervals (helps see pancreas) 16 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 CT – abdominal (cont’d.) • Pros: – More sensitive than US in assessing periampullary region – Can pick up distant mets – Visualize regional lymph nodes, liver, peritoneum, lungs, and bone • Cons: – Inadequate for staging because lacks spatial resolution for invasion of nearby structures – Can not see small ampullary neoplasms • Detection as low as 20% • For patient 1, he subsequently had CTA abdomen & pelvis several days after initial CT abdomen 17 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: CTA abdomen Ampullary mass CBD Stent C+/- coronal CTA abdomen BIDMC, PACS 18 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: CTA abdomen Air in central intrahepatic ducts Pneumobilia C+/- coronal CTA abdomen PANC DUCT MINIP BIDMC, PACS Air in central intrahepatic ducts and pneumobilia secondary to CBD stent placement 19 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 MR and MR cholangiopancreatography (MRCP) • Usually, in patients where ERCP contraindicated • Masses usually appear isointense or hypointense on T1- and T2-weighted images • When mass not seen on MR, bulging duodenal papilla may be only indication of ampullary cancer – Bulging caused by dilated pancreatic and bile ducts • MRCP – noninvasive way to visualize pancreaticobiliary tree • Some signs that differentiate one periampullary cancer from another – So-called “four segment sign” on MR in pancreatic adenocarcinoma 20 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 MR and MR cholangiopancreatography (MRCP) Companion Patient 4 MRCP Coronal T2 From Kim JH et al., Differential Diagnosis of Periampullary Carcinomas at MR, 2002 Axial T2 Hypointense mass 21 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Endoscopic retrograde cholangiopancreatography (ERCP) • Combines endoscopy and fluoroscopy • Visualizes stomach, duodenum, ampulla – Cannot evaluate extent of local tumor invasion • Fluoroscopy with contrast allows for radiographic visualization of bile ducts and pancreatic duct • Diagnostic and therapeutic – – – – Removal of some stones Insertion of stent (retrograde) Dilation of strictures Biopsy • Does have some contraindications, complications 22 John B. Moore, MSc, MS3 Gillian Lieberman, MD Normal ERCP March 2013 Common Hepatic Duct Endoscope Cystic Duct Gallbladder Common Bile Duct Pancreatic Duct Ampulla Duodenum From Greenberger NJ, Blumberg RS, Burakoff R, CURRENT Diagnosis & Treatment, 2nd Edition 23 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: Ampullary lesion on ERCP • Mass was visualized and biopsied. – Stent placed → obstruction relieved BIDMC, ERCP Sphincterotomy with stent placement Ampullary mass From Martin & Moser, Ampullary carcinoma, UpToDate Companion Patient 5 Nodular ampullary carcinoma (reference case) 24 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: ERCP Results (cont’d.) • Fluoroscopy image sequence: – Stent placed → obstruction relieved BIDMC, ERCP Dilated common bile duct on cholangiogram Stricture of distal CBD due to ampullary mass Stricture, obstruction, and dilation relieved Stent being placed 25 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Endoscopic Ultrasound (EUS) • Pros: – Higher spatial resolution than CT/MRI – Can show surrounding anatomy including lymph nodes – Discerns duodenal wall and pancreas interface – More accurate in detecting ampullary tumors than US and CT • As in 100% accurate – FNA ability – Great for preop planning and T-stage • 70-90% accurate in T-stage • Cons: – – – – Technically challenging Operator dependent No stent ability Less adept at nodal-staging in comparison to tumor-staging Especially useful when ERCP has found low-grade dysplasia → Could allow for local resection 26 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Companion Patient 6: Tumor on EUS DL: duodenal lumen T: tumor mass CBD: common bile duct m: muscularis propria nLN: non-metastatic lymph node P: pancreas From Skordilis P et al., Is endosonography an effective method for detection and local staging of the ampullary carcinoma?, 2002 27 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Companion Patient 7 Intraductal ultrasound (IDUS) • Small miniprobes ~2mm • From endoscope into biliary or pancreatic duct • Only modality that can differentiate sphincter of Oddi muscle from papilla • Useful in identifying tumor strictures when no mass seen on imaging or indeterminate strictures – Increases accuracy of ERCP Malignant stricture Companion Patient 8 Benign stricture From: Stavropoulos S et al., Intraductal ultrasound for the evaluation of patients with biliary strictures , 2005 28 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: Diagnosis & Surgery • Cytology sent from ERCP brushings – “Adenomatous mucosa with villous and papillary features, and at least high grade dysplasia” – Path Report BIDMC • Given HGD → surgery – Whipple: pancreaticoduodenectomy – In this case, Robot-assisted, pylorus-preserving 29 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Carcinoma of ampulla of Vater: Some facts • 6-35% of pancreaticoduodenal malignancies – But, rare: 4-6 cases per million people – Average age of diagnosis for sporadic cases → 60-70 • Can be earlier in genetic syndromes, e.g. FAP w/increased risk – Male-to-female ratio 2:1 • Papillary orifice of ampulla commonly involved by tumor – Means symptoms appear early – Abdominal pain, pruritus, obstructive jaundice, steatorrhea, weight loss • Survival is ~25% at 5 years for pts with +LNs and 50% in those without involved nodes – 80% thought to be resectable at Dx 30 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Ampullary carcinoma: location correlates with prognosis → which correlates with histology Large overall size, small invasive component, best overall prognosis. 3-y survival, 73% From: Adsay V, et al., Ampullary Region Carcinomas, 2012 31 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Ampullary carcinoma: location correlates with prognosis → which correlates with histology Largest, highest incidence of LN mets. Minimal intra-amp lumen. Mostly intestinal histology (75%). 3-y survival, 69% From: Adsay V, et al., Ampullary Region Carcinomas, 2012 32 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Ampullary carcinoma: location correlates with prognosis → which correlates with histology Ulcero-nodular tumors, does not show features of other subtypes. Intermediate tumor size. 3-y survival, 54% From: Adsay V, et al., Ampullary Region Carcinomas, 2012 33 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Ampullary carcinoma: location correlates with prognosis → which correlates with histology Smallest but worst prognosis, presumably due to the pancreatic histology or origin (in 86%). 3-y survival, 41% From: Adsay V, et al., Ampullary Region Carcinomas, 2012 34 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Ampullary carcinoma: location correlates with prognosis → which correlates with histology From: Adsay V, et al., Ampullary Region Carcinomas, 2012 35 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Patient 1: Subsequent course • Final path of mass: – Ampullary adenocarcinoma, moderately differentiated, 1+ node of 29 – T1N1 → tumor limited to Ampulla of Vater w/regional LN met. Negative margins • Unfortunately, pancreaticobiliary histology – Cytokeratin 7 (CK7) and CK20 have recently (2013) been shown to differentiate between pancreaticobiliary and intestinal ampullary histology – CK20 → intestinal type; CK7 → pancreaticobiliary type – Patient 1 was CK7+/CK20 – , which is pancreaticobiliary • Now, receiving adjuvant chemo – Still experimental: gemcitabine 1000 mg x2 weekly, 3 weeks on, 1 week off for 4-6 months • He will f/u with surgeon in 3 months for staging 36 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Diagnostic-Therapeutic Algorithm From: Roberts KJ, et al., Endoscopic ultrasound assessment of lesions of the ampulla of Vater, 2013. 37 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Summary • Ampullary masses can be difficult to assess on crosssectional imaging • CT and trans-abdominal US will usually be done to r/o other processes • ERCP is first-line modality for suspected malignant strictures, supplemented by EUS • MR with MRCP and IDUS in special circumstances • Future refinement of radiological modalities to help correlate with new path subdivisions which predict prognosis 38 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 References 1) Adsay V, Ohike N, Tajiri T, et al. Ampullary Region Carcinomas: Definition and Site Specific Classification with Delineation of Four Clinicopathologically and Prognostically Distinct Subsets in an Analysis of 249 Cases. The American Journal of Surgical Pathology 2012; 36(11): 1592-1608. 2) Albores‐Saavedra, Jorge, et al. Cancers of the ampulla of Vater: demographics, morphology, and survival based on 5,625 cases from the SEER program. Journal of surgical oncology 2009; 100(7): 598-605. 3) Carr BI. Chapter 92. Tumors of the Liver and Biliary Tree. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, eds. Harrison's Principles of Internal Medicine. 18th ed. New York: McGraw-Hill; 2012. http://www.accessmedicine.com.ezp-prod1.hul.harvard.edu/content.aspx?aID=9116154. Accessed March 24, 2013. 4) Greenberger NJ, Blumberg RS, Burakoff R. CURRENT Diagnosis & Treatment: Gastroenterology, Hepatology, & Endoscopy, 2nd Edition: www.accessmedicine.com. Accessed 24 March 2013. 5) Kim JH, Kim, MJ, Chung JJ, et al. Differential Diagnosis of Periampullary Carcinomas at MR Imaging. Radiographics 2002; 22(6): 1335-1352. 6) Martin JA, Moser AJ. Ampullary carcinoma: Epidemiology, clinical manifestations, diagnosis and staging. http://www.uptodate.com/contents/ampullary-carcinoma-epidemiology-clinical-manifestations-diagnosis-andstaging?source=search_result&search=ampullary+carcinoma&selectedTitle=2~21#. UpToDate. Accessed 22 March 2013. 7) Morini S, Perrone G, Borzomati D, et al. Carcinoma of the Ampulla of Vater: Morphological and Immunophenotypical Classification Predicts Overall Survival. Pancreas 2013; 42: 60-66. 8) Rivadeneira DE, Pochapin M, Grobmyer SR, et al. "Comparison of linear array endoscopic ultrasound and helical computed tomography for the staging of periampullary malignancies." Annals of surgical oncology (2003; 10(8): 890-897. 9) Roberts KJ, McCulloch N, Sutcliffe R, et al. Endoscopic ultrasound assessment of lesions of the ampulla of Vater is of particular value in low‐grade dysplasia. HPB 2013; 15; 18–23. 10) Skordilis P, Mouzas IA, Dimoulios PD, et al. Is endosonography an effective method for detection and local staging of the ampullary carcinoma? A prospective study. BMC surgery 2002; 2(1): 1-8. 11) Stavropoulos S, Larghi A, Verna E, et al. Intraductal ultrasound for the evaluation of patients with biliary strictures and no abdominal mass on computed tomography. Endoscopy 2005; 37(8): 715-721. 12) Talamini MA, Moesinger RC, Pitt HA, et al. Adenocarcinoma of the ampulla of Vater. A 28-year experience. Annals of surgery 1997; 225(5): 590. 39 John B. Moore, MSc, MS3 Gillian Lieberman, MD March 2013 Acknowledgments • • • • • Dr. Gunjan Senapati Dr. Arthur J. Moser Dr. Gillian Lieberman Claire Odom Victoria Van Voorhees 40