Hereditary Colorectal Polyposis and Cancer Syndromes: A Primer on Diagnosis and Management.

Kanth P1, Grimmett J2,3, Champine M2,3, Burt R1,2,4, Samadder NJ1,2,5.

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Abstract

Colorectal cancer (CRC) is the fourth most common cancer amongst men and women. Between 3 and 6% of all CRCs are attributed to well-defined inherited syndromes, including Lynch syndrome, familial adenomatous polyposis (FAP), MUTYH-associated polyposis (MAP), and several hamartomatous polyposis conditions. Identification of these patients through family history and appropriate genetic testing can provide estimates of cancer risk that inform appropriate cancer screening, surveillance and/or preventative interventions. This narrative review examines the hereditary colorectal cancer and polyposis syndromes, their genetic basis, clinical management, and evidence supporting cancer screening.Am J Gastroenterol advance online publication 8 August 2017; doi:10.1038/ajg.2017.212.