Turcot syndrome is the association between familial adenomatous polyposis and brain tumors like medulloblastoma,malignant glioma. It was first reported by Canadian surgeon Jacques Turcot (1914- ) et al in 1959 and hence carries the first author's name.Turcot J, Després JP, St. Pierre F. Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases. Dis Col Rect 1959;2:465-468. PMID 13839882.

The genetic basis of Turcot syndrome is uncertain.The gene most likely to be involved is APC gene in chromosome 5q.Fauci,et al Harrison's Principle of Internal Medicine 16th Ed. p 2453 However,the syndrome has been claimed to be linked to various mutations in a number of genes. For example, the mismatch repair genes MLH1 () or PMS2 ().

Synonyms

• Brain tumor-polyposis syndrome
• Glioma-polyposis syndrome

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