Small intestine & ampulla


Gangliocytic paraganglioma

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

Topic Completed: 6 January 2021

Minor changes: 12 January 2021

Copyright: 2003-2021,, Inc.

PubMed Search: Gangliocytic paraganglioma ampulla

Raul S. Gonzalez, M.D.
Page views in 2020: 2,576
Page views in 2021 to date: 3,425
Cite this page: Gonzalez RS. Gangliocytic paraganglioma. website. Accessed December 8th, 2021.
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

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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

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Bivalved tumor

Tumor near ampulla

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
Sample pathology report
  • Ampulla, mass, resection:
    • Gangliocytic paraganglioma (2.2 cm) (see comment)
    • Margins of resection unremarkable.
    • Comment: Immunohistochemical stains for S100 and synaptophysin highlight various components of this uncommon triphasic neoplasm, which is typically indolent but can rarely metastasize.
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 style answer #1
B. Epithelioid, ganglion and spindle

Comment Here

Reference: Gangliocytic paraganglioma
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 style 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.

Comment Here

Reference: Gangliocytic paraganglioma
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