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There is limited information on the risk of metachronous colorectal cancer in Lynch syndrome. We reviewed the rate and characteristics of colorectal cancer developing afterprimary oncological bowel resection in a cohort of patients with Lynch syndrome.Follow-up of 32 unrelated patients with Lynch syndrome was reviewed to investigate the development of metachronous colorectal carcinomas. Clinical, pathologic and genetic variables of patients with and without metachronous cancer were compared in order to identify anypredictor of subsequent colorectal cancer risk.One or more metachronous colorectal cancers developed in eight patients (25%) at a median of 2.5 years (range 1-8 years) afterprimary surgery. Cumulatively, 12 patients (37.5%) developed a rectal carcinoma; two of them afterprimary colectomy. When comparing patients with and without metachronous cancer, length and appropriateness of follow-up were similar in both groups. None of the examined clinical, pathologic and genetic factors (including pattern of surgery) was significantly associated with the risk of metachronous cancer.Lynch syndrome is associated with a high risk of metachronous carcinomas both in the colon and in the rectum. Total colectomy with subsequentproctoscopic surveillance may be inadequateprophylactic surgery.