Small bowel (small intestine)
Benign tumors and tumor-like conditions
Gangliocytic paraganglioma

Topic Completed: 1 August 2012

Revised: 13 March 2019

Copyright: 2003-2019,, Inc.

PubMed Search: Gangliocytic paraganglioma[TIAB] small bowel

See also: Ampulla chapter

Hanni Gulwani, M.B.B.S.
Page views in 2019: 2,296
Page views in 2020 to date: 304
Cite this page: Gulwani H. Gangliocytic paraganglioma. website. Accessed February 27th, 2020.
Definition / general
  • Rare tumor of periampullary region and second portion (middle) of duodenum
  • Rarely in jejunum or ileum
  • May derive from endodermal neuroectodermal complexes in the embryonic ventral pancreas, due to totipotential nature of adult stem cells that differentiate along different lineages
Clinical features
  • Presents with GI bleed, abdominal pain or anemia
  • May be multiple or associated with neurofibromatosis 1 or carcinoid syndrome
  • Usually benign, 7% had nodal metastases at presentation (BMC Cancer 2011;11:187), rarely has metastases of endocrine component
Case reports
Clinical images

Images hosted on other servers:

submucosal tumor
and surface ulcer

Gross description
  • Usually 1 - 3 cm, sessile or polypoid
Gross images

Images hosted on other servers:

Tumor near ampulla

Malignant tumor

Microscopic (histologic) description
  • Submucosal lesion(s) with overlying ulcerations
  • Organoid pattern
  • Triphasic with endocrine (carcinoid-like cells in compact nests and trabeculae), spindle cell (Schwann cell-like) and ganglion type cells of varying proportions resembling carcinoid, paraganglioma and ganglioneuroma
  • Variable stromal amyloid
Microscopic (histologic) images

Images hosted on other servers:

Various images

Malignant tumor

Positive stains
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