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Turcot Syndrome (Glioma Polyposis): A Case Report

Sanjay Sarin, MD, Albert Bernath, MD
Volume: 101 Issue: 12 December, 2008

Abstract:

Turcot's syndrome (glioma-polyposis) is a rare hereditary disorder characterized by association of colonic polyposis with primary tumors of the central nervous system. We report a case of a 27-year-old male diagnosed with Turcot's syndrome after an autopsy. The patient survived for more than two decades after his initial presentation with medulloblastoma at the age of five years. Such a long survival is exceptional in patients with this syndrome. Based on the genetic mutations, the patients with Turcot's syndrome are classified into adenomatous polypois coli (APC) group or hereditary non-polyposis colon cancer (HNPCC) group. The article highlights the contrasting features of the two groups.

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References:

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12. Gastrointestinal polyposis syndromes, in Feldman M, Friedman LS, Sleisenger MH (eds): Sleisenger and Fordtran's Gastrointestinal and Liver Disease. Philadelphia: W B Saunders, ed 7, 2002, pp 2197–2203.

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