Colorectal cancer survival advantage in MUTYH-associated polyposis and Lynch syndrome families.

The cardinal features of colorectal cancer in patients with the MUTYH-associated polyposis syndrome, featured in this editorial, are of very recent discovery and remain under review as new clinical phenotypes for these patients are still being identified. Importantly, its phenotype results from homozygosity of the MUTYH gene and, therefore, is inherited in an autosomal recessive pattern. In a more intensively studied hereditary colorectal cancer syndrome, namely Lynch syndrome, the cardinal features have been shown to result from an autosomal dominantly inherited pattern and includes earlier average age of cancer onset, when compared with the general population (45 years for Lynch syndrome colorectal cancer and 64 years for patients with colorectal cancer in the general population), accelerated carcinogenesis, high risk for metachronous colorectal cancers, specific pathology features for Lynch syndrome colorectal cancers that are more often poorly differentiated and have an excess of Colorectal Cancer Survival Advantage in MUTYH-Associated Polyposis and Lynch Syndrome Families