Lynch syndrome, an autosomal dominant multicancer disorder, is the most common form of hereditary colorectal cancer, has a prevalence of 0.9% to 2.7%,1 and also accounts for approximately 2.3% of all endometrial cancers.2 However, patients with Lynch syndrome are frequently unrecognized because family history is often not fully assessed, and patients most commonly have few or no colorectal polyps, as opposed to the more easily identifiable polyposis syndromes. Patients with Lynch syndrome present with cancer at a relatively young age and they are at a higher risk of developing more than 1 type of cancer. The most common extracolonic tumors arise from the endometrium, stomach, ovary, small bowel, and urinary tract.3
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