Turcot syndrome

Editorial Board Member: Raul S. Gonzalez, M.D.
Jennifer Findeis-Hosey, M.D.

Topic Completed: 1 March 2016

Minor changes: 1 October 2020

Copyright: 2002-2020,, Inc.

PubMed Search: Turcot's syndrome [title]

Jennifer Findeis-Hosey, M.D.
Page views in 2019: 690
Page views in 2020 to date: 504
Cite this page: Findeis-Hosey J. Turcot syndrome. website. Accessed October 24th, 2020.
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
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