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Abstract Introduction: Lynch syndrome is an autosomal dominant hereditary cancer syndrome caused by germline mutations in DNA mismatch repair (MMR) genes, conferring elevated risks of colorectal, endometrial, gastric, ovarian and other malignancies. We report a 38-year-old woman with a germline MSH2 c.958dupA (p.Thr320Asnfs*13) pathogenic frameshift mutation who developed metachronous multiple primary neoplasms affecting four organ systems over eight years.
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