Ampulla of Vater
Gangliocytic paraganglioma

Author: Hanni Gulwani, M.D. (see Authors page)

Revised: 22 January2016, last major update November 2012

Copyright: (c) 2003-2016,, Inc.

PubMed Search: Gangliocytic paraganglioma[title]
Cite this page: Gangliocytic paraganglioma. website. Accessed June 22nd, 2018.
Definition / general
  • Rare tumor of periampullary region and second part of duodenum
  • Usually benign, rarely has local metastases of endocrine component but even these cases are indolent
  • May recur if incompletely excised
  • May derive from endodermal-neuroectodermal complexes in the embryonic ventral pancreas
  • Presents with GI bleeding or incidental finding
Case reports
Clinical images

Periampullary submucosal tumor and surface ulcer bleeding.

Gross description
  • Usually 1-3 cm, sessile or polypoid, no capsule
Microscopic (histologic) description
  • Unencapsulated submucosal lesion
  • Triphasic, with epithelioid, spindle cell (Schwann cell like), and ganglion type cells of varying proportions resembling carcinoid tumor (endocrine type cells in compact nests and trabeculae), paraganglioma and ganglioneuroma
  • Usually infiltrative pattern
  • Variable stromal amyloid
Microscopic (histologic) images

Images hosted on other servers:

Various images

Ganglion-like (fig 3), spindle (fig 4A) & epithelial cells (fig 4B)

Positive stains
  • Endocrine cells - pancreatic polypeptide, somatostatin
  • Ganglion cells - chromogranin, synaptophysin, neuron specific enolase, somatostatin
  • Spindle cells - S100
Electron microscopy description
  • Dense core granules
Differential diagnosis
  • Ganglioneuroma
  • Paraganglioma
  • Carcinoid
  • Carcinoma
Additional references