Mismatch repair cancer syndrome

Mismatch repair cancer syndrome
Other namesBrain tumor-polyposis syndrome, Glioma-polyposis syndrome
This condition is inherited in an autosomal recessive manner
SpecialtyOncology

Mismatch repair cancer syndrome (MMRCS) is a cancer syndrome associated with biallelic DNA mismatch repair mutations. It is also known as Turcot syndrome (after Jacques Turcot, who described the condition in 1959) and by several other names.

In MMRCS, neoplasia typically occurs in both the gut and the central nervous system (CNS). In the large intestine, multiple colonic polyps develop; in the CNS, brain tumors.

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