Neuroendocrine neoplasms
Cystic endocrine tumors

Author: Deepali Jain, M.D. (see Authors page)

Revised: 22 December 2017, last major update August 2012

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Cystic endocrine[TI] tumors pancreas[TIAB]

Cite this page: Jain, D. Cystic endocrine tumors. website. Accessed November 21st, 2018.
Definition / general
  • Distinctive subgroup of pancreatic neuroendocrine tumors that are well differentiated, with low mitotic rate and low Ki67 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
Gross images

Images hosted on PathOut server:

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

Images hosted on other servers:

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

Cystic pancreatic
endocrine neoplasm
with glistening

Microscopic (histologic) 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 )
Microscopic (histologic) images

Images hosted on other servers:

20 year old woman:

Cyst wall has collagenous fibrous tissue with small nests of round cells with amphophilic cytoplasm and stippled nuclear chromatin

Tumor cells are synaptophysin+ (a), chromogranin+ (b), NSE+ (c), Ki67 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

Images hosted on other servers:

Conventional smear