Pancreatic Cystic Lesions: an overview

1. Pancreatic Cystic Lesions: an overview Journal Club, 11 Dicembre 2017 Ludovica Venezia

3. Pancreatic Cystic Lesions Prevalence of incidental pancreatic cystic lesions in the adult population ranges from 2.6 to 19.6% Increase of prevalence, size and numbers with age Natural history still partially unclear: some show benign behavior, others have a malignant potential and are precursors of pancreatic ductal adenocarcinoma  importance of correct management Signs/symptoms include: abdominal pain, acute pancreatitis, nausea, vomiting, weight loss, anorexia, exocrine pancreatic insufficiency and steatorrhea, recent onset or worsening diabetes, obstructive jaundice, palpable mass

4. Pseudocyst Initial evaluation of a pancreatic cyst should be directed towards exclusion of pseudocyst Most common cystic lesion of the pancreas (70-75%) A fluid collection of pancreatic juice with a well-defined capsule of fibrous or granulation tissue BUT without an epithelial lining, calcification/debris possible History of acute (necrosis) or chronic (elevated pressure) pancreatitis, rarely abdominal trauma At least 4 weeks after an acute injury

5. Pseudocyst: treatment 50% resolve spontaneously Indication for drainage: symptoms, enlargement > 6 cm, complications (infection, GI obstruction, hemorrhage), suspicion of malignancy Intervention: endoscopic or percutaneous or surgical drainage

6. Cystic Pancreatic Tumors

8. Cystic Pancreatic Tumors All patients with pancreatic cystic neoplasms require a diagnostic work-up: high resolution imaging techniques, such as MRI with MRCP and EUS + FNA in doubtful case Evaluate personal and familial history for pancreatic cancer and/or other malignancies, consistent with Von Hippel–Lindau syndrome (haemangioblastoma, clear cell kidney carcinoma, NETs, pheochromocytoma) Measure and monitor glucose and Ca 19-9 levels No additional examinations are required when the patient, after diagnosis, is found to be unfit for any treatment and asymptomatic

9. Serous Cystadenoma Benign (but 26 cases of cystadenocarcinoma in the literature) 75% W, grandmothers neoplasm Mainly head of the pancreas Lobulated, honeycombed microcystic appearance or polycistic, central scar (with calcification in 20%) Resection only if symptomatic Otherwise, imaging annual follow-up with MRI, glucose, Ca 19.9 until fit for surgery

10. Mucinous Cystic Neoplasm Premalignant lesion 99% W, mothers neoplasm 95% body and tail of the pancreas Septated or uniloculated macrocyst FNA: CEA >192 ng/ml, low amylase Resection ALWAYS! Large size (>4 cm), mural nodules, mass forming lesions or peripheral “egg shell” calcifications suggestive of for invasive malignancy

11. IPMN Intraductal papillary mucinous neoplasm: degenerated cells of the pancreatic ducts producing mucus  pancreatic cysts 10-15% of pancreatic cystic neoplasm M = W, 50-70 years old (main duct earlier than branch) Mandatory: communication with pancreatic duct or branches  FNA: high amylase and mucin Increased risk of ductal adenocarcinoma (main>branch) Synchronous and metachronous extrapancreatic malignant diseases in 20-30% of patients with IPMN

13. IPMN: main duct Segmental or diffuse dilation of MPD > 5 mm without causes of obstruction Prevalence of cancer 60-90%: partial or total pancreasectomy if fit for surgery IPMN mixed type is managed as IPMN main duct High risk stigmata: MPD > 10mm, enhancing mural nodule > 5mm, obstructive jaundice

14. IPMN: branch duct Pancreatic cysts > 5mm that communicate with MPD through branch ducts Mainly head and uncinate process May be multifocal, macro or micro cystic, “bunch of grapes” Typically indolent behavior BUT 5-40% show malignant degeneration Monitor development of high risk stigmata and worrisome features

15. IPMN: branch duct

16. Solid pseudo-papillary neoplasm 1-2% of exocrine pancreatic tumors Young W without significant symptoms Well encapsulated heterogeneous mass with solid and cystic composition, varying amounts of necrosis and hemorrhage Mainly benign and indolent, ~15% can be malignant with MTS at diagnosis Complete resection and lifelong follow-up indicated

17. Cystic Neuroendocrine Tumors Uncommon, often a component of a large NET with cystic degeneration or necrosis Limited data are available for a detailed analysis and for management Less commonly associated with lymph node and liver metastases than solid NETs At imaging: thin, smooth peripheral enhancement > than pancreatic parenchyma.

18. Take Home Messages All cystic lesions require a diagnostic work-up with high resolution imaging (HR-CT, MRI, EUS) Investigate familiar and personal history Evaluate and monitor the presence of worrisome features or high-risk stigmata in order to establish a follow-up vs surgery No additional examinations if unfit for any treatment and asymptomatic A cystic lesion, without suspicious signs, do notexclude the patient from organ transplantation. Otherwise perform CH-EUS+FNA and discuss with surgeon.

19. Bibliografia • Tanaka M, Fernandez-del Castillo C, Adsay V et al. International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology. 2012;12:183–197 • Tanaka M, Fernandez-del Castillo C, Kamisawa T. Revisions of the International Consensus Fukuoka Guidelines for the Management of IPMN of the Pancreas. Pancreatology. 2017;17(5):738-753. • Italian Association of Hospital Gastroenterologists and Endoscopists; Italian Association for the Study of the Pancreas. Italian consensus guidelines for the diagnostic work-up and follow-up of cystic pancreatic neoplasms. Dig Liver Dis. 2014;46(6):479-93. • European Study Group on Cystic Tumours of the Pancreas. European experts consensus statement on cystic tumours of the pancreas. Dig Liver Dis. 2013;45(9):703-11. • Vege S S, Ziring B, Jain R et al. American Gastroenterological Association institute guideline on the diagnosis and management of asymptomatic neoplastic pancreatic cysts. Gastroenterology. 2015;148:819–822