[1] In MMRCS, neoplasia typically occurs in both the gut and the central nervous system (CNS).

[2] Monoallelic mutations of these genes are observed in the condition known as Lynch syndrome or hereditary nonpolyposis colorectal cancer, while biallelic mutations are observed in CMMR-D.[3] People expressing the HNPCC (which itself is considered autosomal dominant) trait are considered carriers of CMMR-D, thus CMMR-D is classified as autosomal recessive.

The polyps developed tend to be larger, fewer, and progress to malignancy earlier than those seen in familial adenomatous polyposis,[1] a clinically similar condition with different underlying mutations.

Turcot syndrome is the association between familial polyposis of the colon and brain tumors[8] like medulloblastoma, malignant glioma.

It was first reported by Canadian surgeon Jacques Turcot (1914-1977 ) et al. in 1959 and hence carries the first author's name.