Table of ContentsDefinition / General | Essential Features | Terminology | Clinical Features | Case Reports | Differential Diagnosis | Additional References
Cite this page: Turcot syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/colontumorturcot.html. Accessed March 24th, 2017.
Definition / General
- Pronounced with silent second t (Turcot was French Canadian)
- Same as FAP or Lynch Syndrome, but additionally with central nervous system tumor (as above)
- 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)
- OMIM: Mismatch Repair Cancer Syndrome (scroll down for information on Turcot)