Turcot syndrome

Overview

What is Turcot syndrome?

Turcot syndrome is a genetic condition that causes growths called polyps in the intestines and a higher chance of getting brain cancer and/or colon cancer. Turcot syndrome is considered an alternative form of two more common cancer syndromes associated with polyp formation- Lynch syndrome and familial adenomatous polyposis syndrome (FAP). Turcot syndrome is also referred to broadly as a mismatch repair deficiency syndrome. Mismatch repair is a process the body uses to repair damage to DNA. Damaged DNA can cause tumors to grow. Using mismatch repair corrects damage done to DNA to prevent a tumor from growing. Sometimes a mutation (change in DNA sequence) causes the parts of the mismatch repair system not to work, which means that it cannot fix other mutations. This leads to a higher chance of tumor development.

SOURCE: Emory University - Department of Human Genetics in collaboration with ThinkGenetic • https://www.thinkgenetic.com/diseases/turcot-syndrome/overview/4796 • DATE UPDATED: 2016-05-27

References

http://www.omim.org/entry/276300

http://www.cancer.net/cancer-types/turcot-syndrome

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