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Colon tumor
Mesenchymal tumors
Ganglioneuromatosis of colon
Reviewers: Charanjeet Singh, M.D. (see Reviewers page)
Revised: 26 March 2012, last major update March 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.
General
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● 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
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● 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
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● Polypoid
● Sessile or pedunculated
Gross images
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Ganglioneuromatous polyposis
Micro description
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● 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
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Diffuse ganglioneuromatosis
Left: polypoid ganglioneuroma; right: ganglioneuromatous polyposis
Positive stains
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● Schwann cells: S100+
● Nerve fibers: NSE+, synaptophysin+, neurofilament+, S100-
● Ganglion cells: c-Ret+, S100+
Molecular description
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● c-RET mutations, even without MEN2b syndrome (J Clin Endocrinol Metab 1998;83:4191)
Differential diagnosis
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● 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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