Abstract

Lynch syndrome is the most common inherited colorectal cancer susceptibility syndrome and accounts for approximately 3 % of all colorectal cancers. Clinical assessment and a detailed family history are crucial in identifying patients who need further evaluation via genetic counseling and testing. Discovery of the underlying causative germline mutations in DNA mismatch repair genes has allowed more accurate colorectal and extracolonic cancer risk assignment. Once diagnosed, intensive surveillance via colonoscopy and timely interventions such as polypectomy reduce colorectal cancer development and mortality from it. If colorectal cancer develops within a Lynch syndrome patient, extended surgical resection is recommended based on high metachronous colorectal cancer risk. Extended resections can be achieved without significant decreases in quality of life. Heightened clinical awareness is needed to promote appropriate diagnosis and management.

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