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WHO Classification of Tumours
Ganglioneuroma
Colon and rectum


Definition

A benign neuroblastic tumor of the sympathetic nervous system that occurs in childhood. Ganglioneuroma typically arises from the sympathetic trunk in the mediastinum. Histologic features include spindle cell proliferation (resembling a neurofibroma) and the presence of large ganglion cells. Common presenting features include a palpable abdominal mass, hepatomegaly, and a thoracic mass detected on routine chest X-ray. Ganglioneuroma features also observed in composite phaeochromocytomas or paragangliomas.

Ganglioneuroma of the colon and rectum
Most gastrointestinal ganglioneuromas occur in the colon with a predilection to the left side and the rectum, and a few are seen in the appendix. They are seen at a wide range of ages and equally in men and women. The usual presentation is a small mucosal polyp of < 12 cm, or sometimes multiple polyps. They are usually sporadic and indolent. However, diffuse murally extending ganglioneuromas (ganglioneuromatosis) have a high association with neurofibromatossis type 1 (NF1) and multiple endocrine neoplasia type 2b (MEN2B) syndromes

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Carney JA, Go VL, Sizemore GW, Hayles AB (1976)
Alimentary-tract ganglioneuromatosis. A major component of the syndrome of multiple endocrine neoplasia, type 2b.
N Engl J Med 295: 1287-91




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Shekitka KM, Sobin LH (1994)
Ganglioneuromas of the gastrointestinal tract. Relation to Von Recklinghausen disease and other multiple tumor syndromes.
Am J Surg Pathol 18: 250-7



. Histologically, there are large numbers of mucosal ganglion cells and spindled Schwann cells, often with numerous eosinophilic granulocytes. Cystic glands can be present, imparting a resemblance to juvenile polyp in some cases. Immunohistochemically, the ganglion cells are positive for neuron-specific enolase (NSE), neurofilaments, and synaptophysin, whereas the Schwannian components are positive for S100 protein and often also for glial fibrillary acidic protein (GFAP)
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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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