Colon (tumor & nontumor)
Mesenchymal tumors

Topic Completed: 1 October 2016

Minor changes: 31 July 2020

Copyright: 2003-2019,, Inc.

PubMed Search: Ganglioneuromatosis colon

Raul S. Gonzalez, M.D.
Page views in 2019: 2,289
Page views in 2020 to date: 1,123
Cite this page: Gonzalez R. Ganglioneuromatosis. website. Accessed August 6th, 2020.
Definition / general
  • Benign hamartomatous proliferation involving the colonic muscularis propria
  • Often associated with MEN IIb (RET mutations) and neurofibromatosis type 1 (NF1 mutations) (Am J Surg Pathol 1994;18:250, Gut 1999;45:143)
Essential features
  • Hamartomatous increase of glial and neuronal elements in the muscularis propria
  • Has syndromic associations
  • Must be distinguished from solitary ganglioneuromas and polypoid ganglioneuromatosis
  • Also called transmural intestinal ganglioneuromatosis or diffuse intestinal ganglioneuromatosis
Clinical features
  • Patients may have diarrhea or intestinal pseudo-obstruction
Radiology description
Case reports
Clinical images

Images hosted on other servers:

Small bowel follow through showing luminal narrowing

Gross description
  • Poorly demarcated mural thickening, up to 17 cm in length, which can distort surrounding tissue
Gross images

Case of the Week #380

Images hosted on other servers:

Ganglioneuromatous polyposis

Microscopic (histologic) description
  • Proliferation of neural cells and ganglion cells in muscularis propria and sometimes in other layers
  • No evidence of malignancy
Microscopic (histologic) images
Contributed by Raul S. Gonzalez, M.D.

Low power view highlighting mucosal disease

Low power view showing mural disease

Medium power view of mucosal disease

Medium power view
showing ganglion and
spindled proliferation
in muscularis propria

High power view
showing mural
disease with
numerous ganglion cells

Case of the Week #305

Solitary ganglioneuroma

Case of the Week #380

Various images

Images hosted on other servers:

Diffuse ganglioneuromatosis

Left: polypoid ganglioneuroma; right: ganglioneuromatous polyposis

Positive stains
Molecular / cytogenetics description
Differential diagnosis
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