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

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

Revised: 19 February 2018, last major update August 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Gangliocytic paraganglioma[TIAB] small bowel

See also: Ampulla chapter
Cite this page: Gulwani, H. Gangliocytic paraganglioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/smallbowelgangliocyticpara.html. Accessed October 19th, 2018.
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:

Periampullary
submucosal tumor
and surface ulcer
bleeding

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