Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Ganglioneuroma

  • Berna Savaş
  • Arzu Ensari
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-40560-5_1464

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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References and Further Reading

  1. 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.PubMedCrossRefGoogle Scholar
  2. 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.PubMedCrossRefGoogle Scholar
  3. 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.PubMedCrossRefGoogle Scholar
  4. Smith, V. V., Eng, C., & Milla, P. J. (1999). Intestinal ganglioneuromatosis and multiple endocrine neoplasia type 2B: Implications for treatment. Gut, 45, 143–146.PubMedPubMedCentralCrossRefGoogle Scholar
  5. 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.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of PathologyAnkara University Medical SchoolAnkaraTurkey
  2. 2.Department of PathologyAnkara University Medical SchoolSihhiyeTurkey