Turcot syndrome


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Tur·cot syn·drome

(tur-kō'), [MIM*276300]
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.

Tur·cot syn·drome

(tur-kō'), [MIM*276300]
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.

Turcot syndrome

A widely used synonym for what is preferentially termed mismatch repair cancer syndrome, see there.

Turcot syndrome

Oncology A syndrome characterized by multiple colon polyps and brain tumors–eg, ependymoma, glioblastoma, medulloblastoma

Turcot,

Jacques, Canadian surgeon, 1914–.
Turcot syndrome - a rare and distinctive form of multiple intestinal polyposis associated with brain tumors.
References in periodicals archive ?
As a differential diagnosis, intestinal polyposis syndromes represent different types of FAP, including hereditary-mixed polyposis syndrome, Hamartomatous polyposis syndromes, and other variants such as Turcot syndrome.
These include Gorlin syndrome and the less common Turcot syndrome, which consists of medulloblastomas in conjunction with familial adenomatous polyposis and adenomatous polyposis coli [2].
These include Turcot syndrome, an inherited condition in which people are at an increased risk of adenomatous polyps (and thus, colon cancer) and brain tumors, and Peutz-Jeghers syndrome, a condition that leads to freckles around the mouth and sometimes on the hands and feet, as well as large polyps in the digestive tract and an increased risk of colon and other cancers at a young age.
FAP has several variant forms, including Turcot syndrome and Garden syndrome, which have other manifestations (e.
Turcot Syndrome was originally described as a rare variant of FAP, in which brain tumors develop concurrently with those of the colon and rectum.
It is an advocacy group for families who have any one of the inherited forms of colon cancer, which include familial adenomatous polyposis or Gardner syndrome (FAP/GS), juvenile polyposis (JP), Peutz-Jeghers syndrome, hereditary non-polyposis colon cancer (HNPCC/Lynch syndrome) and Turcot syndrome.