Small intestine & ampulla
Gangliocytic paraganglioma

Editor-in-Chief: Debra Zynger, M.D.
Raul S. Gonzalez, M.D.

Topic Completed: 12 December 2018

Minor changes: 11 February 2020

Copyright: 2003-2019,, Inc.

PubMed Search: Gangliocytic paraganglioma ampulla

See Also: Small bowel, Esophagus, Nasal cavity (tumor at these sites)

Raul S. Gonzalez, M.D.
Page views in 2019: 802
Page views in 2020 to date: 1,737
Cite this page: Gonzalez RS. Gangliocytic paraganglioma. website. Accessed September 29th, 2020.
Definition / general
  • Rare tumor of periampullary region and second part of duodenum
Essential features
  • Duodenal neoplasm that rarely metastasizes
  • Triphasic histology (epithelioid, spindle, ganglion type cells) and immunoprofile are characteristic and distinctive
ICD coding
  • ICD-10: D13.2 - Benign neoplasm of duodenum
Clinical features
  • Tissue diagnosis required
Radiology images

Images hosted on other servers:

CT scan

Prognostic factors
  • Almost always benign but distant metastases can rarely occur
Case reports
Clinical images

Images hosted on other servers:

Endoscopic polypoid lesion

Periampullary submucosal tumor

Gross description
  • Usually 1 - 3 cm, sessile or polypoid, no capsule
Gross images

Images hosted on other servers:

Bivalved tumor

Tumor near ampulla

Malignant tumor

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Raul S. Gonzalez, M.D.

Submucosal tumor

Nests and trabeculae

Epithelioid cells

Spindled cells

Ganglion cells

Cytology description
Positive stains
Molecular / cytogenetics description
Differential diagnosis
Board review style question #1
Which of the following cell types are seen in gangliocytic paraganglioma?

  1. Blister, ganglion and stem
  2. Epithelioid, ganglion and spindle
  3. Epithelioid, ganglion and sustentacular
  4. Epithelioid, stem and spindle
Board review answer #1
B. Epithelioid, ganglion and spindle

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Board review style question #2
The following picture shows a lesion resected from the ampulla. Which of the following is true?

  1. Similar tumors may rarely be seen outside the ampulla
  2. The patient is at high risk for distant metastasis
  3. The patient likely has MEN1 syndrome
  4. This lesion usually shows translocations involving EWS
Board review answer #2
A. Similar tumors may rarely be seen outside the ampulla. The picture shows a gangliocytic paraganglioma, which usually occurs in the ampulla but can rarely arise in other gastrointestinal or extragastrointestinal sites.

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