Turcot syndrome – see Lynch syndrome

Turcot syndrome

Turcot syndrome is a clinical syndrome used to describe families which had both bowel (colon) cancers and brain tumours.

It's an old term, first used in the 1950s by Mr Jacques Turcot, a surgeon. (Turcot is pronounced Tur coh, the "t" is silent).

It is no longer used as it describes the clinical features of 2 hereditary cancer syndromes that have very different genetic causes and different cancer risks: Lynch syndrome and Familial Adenomatous Polyposis syndrome.

In Lynch syndrome, there may be only a few bowel polyps but the lifetime risk of colon cancer is more than 30 or 40% without screening. The brain tumours were usually glioblastoma.

In Familial Adenomatous Polyposis syndrome, hundreds or even thousands of polyps may occur in the bowel from a very young age and the lifetime risk of colon cancer is 100% if nothing is done to reduce the risk. The brain tumours are usually medulloblastoma.

Read more about Lynch syndrome and Familial Adenomatous Polyposis syndrome.