Colon and rectum
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 < 1–2 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)
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
Shekitka KM, Sobin LH (1994)
. 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)
Ganglioneuromas of the gastrointestinal tract. Relation to Von Recklinghausen disease and other multiple tumor syndromes.
Am J Surg Pathol 18: 250-7
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
International Agency for Research on Cancer: Lyon 2010