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Neuroendocrine tumors

Cystic endocrine tumors

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 1 December 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.


● Distinctive subgroup of pancreatic neuroendocrine tumors that are well differentiated, with low mitotic rate and low Ki-67 proliferation index (Am J Surg Pathol 2012;36:1666)
● Up to 17% of pancreatic endocrine tumors are purely or partially cystic (J Am Coll Surg 2008;206:1154), due to cystic degeneration of solid endocrine tumors

Clinical features

● Mean 5 cm, usually nonfunctional
● 25% are associated with MEN1: occur in younger patients, often multiple, in tail and express glucagon (Virchows Arch 2011;458:47)
● By imaging have peripheral hypervascular rim and images of cyst into cyst
● Confirm by octreoscan scintigraphy
● Excise since may be malignant
● Prognosis good

Case reports

● 20 year old woman with incidental tumor (J Cancer Res Ther 2012;8:289)

Gross images

Cut surface of an 8 cm, expansile endocrine tumor with cystic pattern

Large spherical lesion arising from head of pancreas, cyst has multiple loculations

Large cystic pancreatic endocrine neoplasm with glistening capsule and septated appearance

Micro description

● Thick cyst wall with nests of neuroendocrine cells
● Also neuroendocrine cells grouped in vesicular structures surrounded a fluid filled cavity (Am J Surg Pathol 2001;25:752)

Micro images

20 year old woman:
Left: cyst wall has collagenous fibrous tissue with small nests of round cells with amphophilic cytoplasm and stippled nuclear chromatin
Right: tumor cells are synaptophysin+ (a), chromogranin+ (b), NSE+(c), Ki-67 low (d), CK19- (e), CD10-(f)

Cytology description

● Loosely cohesive aggregates and single cells; cells are small, plasmacytoid, with occasional cytoplasmic vacuoles; nuclei are round/oval, uniform, with finely and evenly distributed chromatin (Cancer 2009;117:203)

Cytology images

conventional smear

End of Pancreas > Neuroendocrine tumors > Cystic endocrine tumors

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