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  • Title: Turcot syndrome (glioma polyposis): a case report.
    Author: Sarin S, Bernath A.
    Journal: South Med J; 2008 Dec; 101(12):1273-4. PubMed ID: 19005436.
    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 polyposis 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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