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.

References
  • http://www.omim.org/entry/276300
  • http://www.cancer.net/cancer-types/turcot-syndrome
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Are there other names for Turcot syndrome?

Are there other names for Turcot syndrome?

Turcot syndrome is also known as Brain Tumor-Polyposis Syndrome 1 (BTPS1) or BTP1 Syndrome. Turcot syndrome is also referred to broadly as a mismatch repair deficiency syndrome. This is a category that includes other genetic syndromes. 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).

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

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