Turcot syndrome: report of a case and review of the literature.

Abstract

We report a case of Turcot's syndrome in a 20-year old man with multiple adenomatous polyps of the colon and glioblastoma multiforme. Detailed histopathological study of all 25 polyps removed from his colon confirmed the distinct morphological and numerical features of the colonic polyposis in Turcot's syndrome. Moreover, 45% of the total polyps and all polyps with a diameter exceeding 2 cm showed malignant transformation, indicating the precancerous nature of these polyps. These findings are discussed together with data obtained from a literature review of 32 histopathologically confirmed cases of Turcot's syndrome with reference to the possible heterogeneous nature of the syndrome at the present time. The current views on the relationship of Turcot's syndrome to other polyposis coli syndromes are presented.