Turcot syndrome

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.^[1]

The genetic basis of Turcot syndrome is uncertain.The gene most likely to be involved is APC gene in chromosome 5q.^[2] However, the syndrome has been claimed to be linked to various mutations in a number of genes. For example, the mismatch repair genes MLH1 (Online Mendelian Inheritance in Man (OMIM) 120436) or PMS2 (Online Mendelian Inheritance in Man (OMIM) 600259).

• Brain tumor-polyposis syndrome
• Glioma-polyposis syndrome

• Gardner syndrome is an association of hereditary intestinal polyps and osteomas.

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