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Turcot syndrome

Pronunciation: tur-ko'

Definition: a rare and distinctive form of multiple intestinal polyposis associated with brain tumors; autosomal recessive inheritance, caused by mutation in one of the mismatch repair genes: either MLH1 on chromosome 3p, PMS2 on chromosome 7p, or the adenomatous polyposis coli gene (APC) on 5q.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.

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