* Colon tumor - Ganglioneuromatosis of colon

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Colon tumor

Mesenchymal tumors

Ganglioneuromatosis of colon

Reviewers: Charanjeet Singh, M.D. (see Reviewers page)
Revised: 27 March 2014, last major update March 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.


● Either polypoid ganglioneuromas, ganglioneuromatous polyposis or diffuse ganglioneuromatosis
● Usually arises from nerves within bowel wall
● Adults may have polyps, but usually a microscopic diagnosis (Gastroenterol Clin Biol 2003;27:219)
● Low malignant potential

Polypoid ganglioneuromas:
● Small, solitary / few
● Ganglion cells in nests in submucosa or mucosa, usually relatively normal surrounding tissue

Ganglioneuromatous polyposis:
● More than 20 lesions, each with greater variability in ganglionic, neural and stromal content

Diffuse ganglioneuromatosis:
● Proliferation of neural elements involving entire enteric plexus (Ann Pathol 2004;24:129)
● Large lesions up to 17 cm, poorly demarcated, which can distort surrounding tissue
● May cause diarrhea
● Associated with neurofibromatosis type 1 (NF-1 mutation), MEN 2b/3 (RET mutation, Am J Surg Pathol 1994;18:250, Gut 1999;45:143) or Cowden’s disease (PTEN mutation)

Case reports

● 63 year old man with prior tubular adenoma and new colon polyp (Case of the Week #305)
● 77 year old woman with mucinous adenocarcinoma and hyperparathyroidism (Eur J Gastroenterol Hepatol 1999;11:447)
● Causing chronic diarrhea (Ugeskr Laeger 1998;160:7139)

Gross description

● Polypoid
● Sessile or pedunculated

Gross images

Ganglioneuromatous polyposis

Micro description

● Proliferation of ganglion cells and spindled Schwann cells in lamina propria and deeper layers
● May see accentuation of submucosal and myenteric plexus in MEN 2b/3

Micro images

Diffuse ganglioneuromatosis

Left: polypoid ganglioneuroma; right: ganglioneuromatous polyposis

Case of the Week #305 -
Solitary ganglioneuroma

Positive stains

● Schwann cells: S100+
● Nerve fibers: NSE+, synaptophysin+, neurofilament+, S100-
● Ganglion cells: c-Ret+, S100+

Molecular description

● c-RET mutations, even without MEN2b syndrome (J Clin Endocrinol Metab 1998;83:4191)

Differential diagnosis

● Crohn’s disease (AJR Am J Roentgenol 2004;182:1166)
● Intestinal neuronal dysplasia

End of Colon tumor > Mesenchymal tumors > Ganglioneuromatosis of colon

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