Colon tumor
Mesenchymal tumors
Diffuse ganglioneuromatosis of colon

Author: Raul S. Gonzalez, M.D. (see Authors page)

Revised: 18 October 2016, last major update October 2016

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed Search: Ganglioneuromatosis colon
Cite this page: Ganglioneuromatosis of colon. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/colontumorganglioneuromatosis.html. Accessed December 10th, 2016.
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
Terminology
  • 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
Images hosted on PathOut server:

Case of the Week #380



Images hosted on other servers:

Ganglioneuromatous polyposis

Micro Description
  • Proliferation of neural cells and ganglion cells in muscularis propria and sometimes in other layers
  • No evidence of malignancy
Micro Images
Images hosted on PathOut server:

Images contributed by Raul S. Gonzalez, MD:

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 numer-
ous ganglion cells


Case of the Week #380:



Case of the Week #305:

Solitary ganglioneuroma



Images hosted on other servers:

Diffuse ganglioneuromatosis

Left: polypoid ganglioneuroma; right: ganglioneuromatous polyposis

Positive Stains
Molecular / Cytogenetics Description
Differential Diagnosis