Title: Diffuse Ganglioneuromatosis of Colon Presenting as Acute Intestinal Obstruction: A Rare Case Report
Authors: Biparnak Haldar, Saswati Majumdar, Malay Sarkar, Urvee Sarkar
DOI: https://dx.doi.org/10.18535/jmscr/v5i11.02
Abstract
Ganglioneuromas are slow growing, well differentiated neuroectodermal neoplasms, typically derived from the sympathetic ganglia and adrenal glands and more common in children. Ganglioneuromas most frequently occur in the posterior mediastinum, retroperitoneum and adrenal glands but are rare in the colon. Colonic Ganglioneuromas fall into three subgroups – polypoid ganglioneuromas, ganglioneuromatous polyposis and diffuse ganglioneuromatosis. Diffuse ganglioneuromatosis is either transmural or mucosal. Adults tend to have mucosal lesions while children can have both mucosal or transmural lesions. Here we are reporting a case of diffuse ganglioneuromatosis of colon (transmural type), clinically presenting as acute intestinal obstruction in a 79 year old male patient, which is a rare entity.
Keywords- Ganglioneuromas, Diffuse ganglioneuromatosis, colonic ganglioneuroma.
References
- Tae-Jun Kim, Hyun Lim, Ho Suk Kang, Sung Hoon Moon, Jong Hyeok Kim, Choong Kee Park, Mi Jung Kwon, and Bong Hwa Lee. Diffuse Ganglioneuromatosis of the Colon Presenting as a Large Subepithelial Tumor in Adults: Report of Two Cases. Korean J Gastroenterol 2015; 66:111-115.
- George Abraham and Sateesh R. Prakash. Solitary Colonic Ganglioneuroma: Case Reports in Gastrointestinal Medicine, vol. 2015, Article ID 794985, 3 pages, 2015.
- Sridhar R. Charagundla, Marc S. Levine, Drew A. Torigian, Mical S. Campbell, Emma E. Furth, John Rombeau. Diffuse Intestinal Ganglioneuromatosis Mimicking Crohn’s Disease. American Journal of Roentgenology 2004.182:1166-1168.
- Shekitka KM, Sobin LH. Ganglio-neuromas of the gastrointestinal tract. Relation to Von Recklinghausen disease and other multiple tumor syndromes. Am J Surg Pathol 1994; 18:250-257.
- Chambonnière ML, Porcheron J, Scoazec JY, Audigier JC, Mosnier JF. Intestinal ganglioneuromatosis diagnosed in adult patients. Gastroenterol Clin Biol 2003;27:219-224.
- Mateş I, Iosif C, Dinu D, Constantinoiu S. Solitary ganglioneuromatosis of the descending colon, presenting as giant retroperitoneal tumour.Chirurgia (Bucur) 2013; 108:584-588.
- Miettinen M, Fletcher CDM, Kindblom LG, Tsui WMS. Mesechimal tumors of the colon and rectum. In: Bosman FT, Carneiro F, Hruman RH, Theise ND, eds. WHO classification of tumours of the digestive system. 4th ed. Lion: IARC, 2010:140-141.
- M. Shekitka and L. H. Sobin, “Ganglioneuromas of the gastrointestinal tract: relation to Von Recklinghausen disease and other multiple tumor syndromes,” The American Journal of Surgical Pathology, vol. 18, no. 3, pp. 250–257, 1994.
- -L. Chambonni`ere, J. Porcheron, J.-Y. Scoazec, J.-C.Audigier and J.F.Mosnier, “Intestinal ganglioneuromatosis diagnosed in adult patients,” Gastroenterologie Clinique et Biologique, vol. 27, no. 2, pp. 219–224, 2003.
- Carney JA, Go VL, Sizemore GW, Hayles AB. Alimentary-tract ganglioneuro-matosis. A major component of the syndrome of multiple endocrine neoplasia, type 2b. N Engl J Med 1976;295:1287-1291.