Asif Khalid, MD, FACG ACG Regional Postgraduate Course June 6-8, 2014. Washington, DC “Pancreatic Cysts What is Benign versus Not-so-benign” Asif Khalid MD Associate Professor of Medicine University of Pittsburgh Medical Center Chief GI Section, VA Pittsburgh Healthcare System GOALS  What is the differential diagnosis for a pancreatic cyst?  What are the important details you need to know about common pancreatic cystic neoplasms?  How do we differentiate between the different pancreatic cysts?  How do we manage a suspected pancreatic cystic neoplasms? ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 1 Asif Khalid, MD, FACG Pancreatic Cyst No Epithelial Lining Epithelial Lining Pseudocyst Benign Premalignant Serous cystic tumors Retention cysts Rare developmental cysts Solid pseudopapillary neoplasm MCN IPMN Malignant Cystic endocrine tumors Solid tumors with cystic degeneration Mucinous cystadenocarcinoma IPMCarcinoma WHO CLASSIFICATION Pancreatic cystic neoplasms  Serous cystic tumors (SCT) micro and oligo-cystic, oligo-cystic serous cystadenocarcinoma  Mucinous cystic neoplasms (MCN) adenoma, mod dysplasia, carcinoma +/- invasion  Intraductal papillary mucinous neoplasms (IPMN) adenoma, mod dysplasia, carcinoma +/- invasion  Solid pseudopapillary neoplasms (SPN) neoplasm and carcinoma ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 2 Asif Khalid, MD, FACG Serous cystic tumors (SCT)  Occur in both sexes (F>M)  Benign (essentially)  Microcystic and oligocystic variety  Microcystic variant common, honeycomb appearance  May have central scar  Usually asymptomatic  FNA FNA- thin and usually bloody  Cytology- Glycogen rich cuboidal cells  Low CEA (<5 ng/mL)  Mutation on 3p (VHL)  Resect if symptomatic Mucinous cystic neoplasms (MCN)  Occur in women only  Pre-malignant lesion  Septated or unilocular; body, tail location  Egg shell calcification ~20%  Risk of cancer >4cm  Cytology- sensitivity 35%, specificity 80%  CEA elevated (>200 ng/mL ~80% accurate)  KRAS mutation not sensitive (spec. ?)  Ovarian stroma requisite for pathological diagnosis  Resection recommended ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 3 Asif Khalid, MD, FACG Intraductal papillary mucinous neoplasm (IPMN)  Occur in both sexes, Premalignant lesion  Main duct IPMN  Symptoms, Symptoms duct diameter >10mm >10mm, mural nodule associated with malignancy  High risk, Resection recommended  Branch duct IPMN  Size over 3cm, solid component, symptoms, main duct dilation (mixed type) associated with malignancy  Low risk, main management dilemma, resect or survey, and how Cytology- sensitivity 35%, specificity 80% CEA elevated (>200 ng/mL ~80% accurate) KRAS and GNAS mutation specific, not sensitive Clinical Dilemmas  Differentiating bd-IPMN (especially if single lesion) and MCN from retention cysts, oligocystic SCT, and sometimes pseudocysts.  Management of presumed bd-IPMN and by default MCN ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 4 Asif Khalid, MD, FACG Diagnostic Tools  History - Pancreatitis: Timing and correlation with lesion. - Symptoms: Pain, Pain wt. wt loss, loss steatorrhoea, steatorrhoea jaundice. jaundice - Family history of PDC. - DM.  Imaging (CT, MR, EUS) - What is the question? - Local expertise. - Duct vs. parenchyma. - Risks (radiation, sedation) vs. benefit (information). - ERP has very limited role. Diagnostic Tools Illustrative example • 60YOBM comes in with complains of abdominal discomfort, greasy stools and 20lb wt. loss. He drinks beer heavily on the weekends. His CT scan shows What is the differential? • BUT what if you found out he had severe acute pancreatitis 6 months ago and a CT from that time showed now what do you think? ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 5 Asif Khalid, MD, FACG Diagnostic Tools  Aspiration – Cytology less accurate (~50%) than CEA for IPMN and MCN. – Cyst fluid CEA (>200) helpful (~80% accurate) in diagnosing MCN and IPMN. – KRAS/GNAS specific for IPMN – Amylase typically high in PP but can be high in IPMN and even MCN To aspirate or not? What to send the aspirate for? Golden Rules  Take a good history and review ALL imaging to differentiate PP vs. PCN, identify progression.  Imaging cannot differentiate between benign and malignant pancreatic cysts in the absence of a mass.  YOU cannot always trust a imaging “report”.  Cyst fluid CEA helpful in diagnosing MCN and IPMN, KRAS/GNAS in IPMN, BUT may not alter management e.g. - 15mm simple asymptomatic cyst - 3cm symptomatic cyst with solid component ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 6 Asif Khalid, MD, FACG Approach to presumed bd-IPMN Adapted and modified from Sendai criteria <1cm 1-3cm >3cm EUS/FNA + MRCP MRCP 1yr High risk features No <1cm Yes 1-3 cm High risk features Dilated main duct Mural nodule Malignant cytology FH PDC Symptoms MRCP+/-MRI 1-2cm---q6-12 mo 2-3cm---q3-6 mo No High risk features >3cm Yes Resection CASE 1 80YOWF incidentally found to have a large mass in the head of the pancreas while undergoing work up for vaginal bleeding. She denies abdominal pain, wt. loss, h/o pancreatitis, FH of PDC. She also c/o constipation. Choices: 1: This is a PP and will get symptomatic, arrange cystgastrostomy. 2: Proceed with an ERCP to evaluate for duct communication and brushings. 3: This is likely a serous cystic tumor. May consider EUS for further evaluation. 4: Refer for whipple due its size and risk of cancer. ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 7 Asif Khalid, MD, FACG CASE 2 35YOWF found to have a 4cm mass in the tail of the pancreas on CT to evaluate renal colic. She occasionally has dull ache LUQ. She denies wt. loss, h/o pancreatitis, FH of PDC, ETOH or tobacco use. Choices: 1: This is a PP and is symptomatic, arrange cystgastrostomy. 2: Consider an ERCP and stent to help drain it trans-papillary. 3: This is likely a MCN. Either proceed with resection or may consider EUS for confirmation. 4: Obtain an MRCP in 6 months to document stability in this obvious bdIPMN. CASE 3 32YOWF is diabetic and in her second trimester. TAUS showed a lesion in the tail of the pancreas. Her PCP ordered an MRCP and now refers to you you. She c/o heartburn, but denies h/o pancreatitis. Her father died of PDC. MRCP: Cluster of cysts ~15mm TOP communicating with the MPD. Choices: 1: She has IPMN and FH of PDC, proceed with surgery immediately. 2: Schedule distal pancreatectomy after she delivers. 3: Schedule a follow up with you after she delivers, tentatively plan for EUS. 4: Obtain an MRCP in 1 year to document stability in her IPMN. ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 8 Asif Khalid, MD, FACG Suggested Reading • Tanaka M, Fernandez-del Castillo C, Adsay V, Chari S, Falconi M, Jang JY, et al. International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology. 2012;12:183-97. • Khalid A, Brugge W. ACG Practice Guidelines for the Diagnosis and Management of Pancreatic Cysts. Am J Gastroenterology. 2007;102(10):2339-49 ACG Eastern Regional Postgraduate Course - Washington, DC Copyright 2014 American College of Gastroenterology 9