Cancer risk in Lynch Syndrome

Lynch Syndrome, or hereditary non-polyposis colorectal cancer (HNPCC) is an autosomal dominant cancer predisposition syndrome caused by inactivating mutations in DNA mismatch repair genes. It accounts for 2–4 % of all incident colorectal cancers. Mutation carriers are at risk of early onset colorect...

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Bibliographic Details
Published inFamilial cancer Vol. 12; no. 2; pp. 229 - 240
Main Authors Barrow, Emma, Hill, James, Evans, D. Gareth
Format Journal Article
LanguageEnglish
Published Dordrecht Springer Netherlands 01.06.2013
Springer Nature B.V
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Summary:Lynch Syndrome, or hereditary non-polyposis colorectal cancer (HNPCC) is an autosomal dominant cancer predisposition syndrome caused by inactivating mutations in DNA mismatch repair genes. It accounts for 2–4 % of all incident colorectal cancers. Mutation carriers are at risk of early onset colorectal cancer, endometrial cancer, and a spectrum of other tumours. Accurate estimation of cancer risk for mutation carriers is essential for counselling, and establishing appropriate screening guidelines. This study reviews the current data on cancer risk, and emerging risk reduction strategies.
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ISSN:1389-9600
1573-7292
DOI:10.1007/s10689-013-9615-1