Colon tumor
Familial polyposis syndromes
Turcot syndrome

Author: Jennifer Findeis-Hosey, M.D. (see Authors page)
Editorial Board review: Raul Gonzalez, M.D.

Revised: 4 April 2016, last major update March 2016

Copyright: (c) 2002-2016,, Inc.

PubMed Search: Turcot's syndrome[title]

Cite this page: Turcot syndrome. website. Accessed October 24th, 2016.
Definition / General
Essential Features
  • Pronounced with silent second t (Turcot was French Canadian)
Clinical Features
  • Same as FAP or Lynch Syndrome, but additionally with central nervous system tumor (as above)
Case Reports
Differential Diagnosis
  • Constitutional mismatch repair deficiency syndrome: biallelic mismatch repair gene mutation; patients develop brain tumors, hematopoietic malignancies and Lynch syndrome-associated tumors (Haematologica 2010;95:699)
Additional References