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, 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 not exclude 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):
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):
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):
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