Synonyms
Diffuse ganglioneuromatosis; Ganglioneuromatosis; Ganglioneuromatous polyposis; Isolated (solitary) polypoid ganglioneuroma; Mucosal ganglioneuroma
Definition
Ganglioneuroma (GN) of gastrointestinal tract is an extremely rare neuroectodermal tumor that is characterized by the presence of mucosal ganglion cells and spindled Schwann cells often with numerous eosinophils. Gastrointestinal GNs are divided into three groups defined as polypoid GN, ganglioneuromatous polyposis (GP), and diffuse ganglioneuromatosis (DG). The most common form is polypoid GNs form which show a mixture of ganglion cells and nerve fibrils within mucosa, extending to submucosa. In GP, most polyps resemble typical polypoid GN, whereas others show filiform mucosal projections containing groups of ganglion cells with little neural stroma. GP affects patients with familial adenomatous polyposis (FAP), Cowden’s disease, tuberous sclerosis, multiple endocrine neoplasia type 2b (MEN type 2b) syndrome, colorectal...
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Al-Daraji, W. I., Abdellaoui, A., & Salman, W. D. (2005). Solitary polypoidal rectal ganglioneuroma: A rare presentation of a rare tumor. Journal of Gastroenterology and Hepatology, 20, 961–971.
Hechtman, J. F., & Harpaz, N. (2015). Neurogenic polyps of the gastrointestinal tract: A clinicopathologic review with emphasis on differential diagnosis and syndromic associations. Archives of Pathology and Laboratory Medicine, 139(1), 133–9.
Sayki Arslan, M., Ekız, F., Yilmaz, G., Çoban, Ş., Savaş, B., Ensarı, A., & Örmecı, N. (2012). Ganglioneuromatous polyposis of the colon in a patient with multiple adenomatous polyps. The Turkish Journal of Gastroenterology, 23(6), 780–3.
Smith, V. V., Eng, C., & Milla, P. J. (1999). Intestinal ganglioneuromatosis and multiple endocrine neoplasia type 2B: Implications for treatment. Gut, 45, 143–146.
Vinitsky, A., Zaleski, C. A., Sajjad, S. M., & McPherson, E. W. (2013). Intestinal ganglioneuromatosis: Unusual presentation of Cowden syndrome resulting in delayed diagnosis. American Journal of Medical Genetics Part A, 161A(5), 1085–90.
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Savaş, B., Ensari, A. (2017). Ganglioneuroma. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1464
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1464
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