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Cancer risk in Lynch Syndrome

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Abstract

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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Abbreviations

HNPCC:

Hereditary non-polyposis colorectal cancer

MMR:

Mismatch repair

ICG-HNPCC:

International Collaborative Group on HNPCC

CRC:

Colorectal cancer

CNS:

Central nervous system

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Acknowledgments

DGE is supported by the Manchester NIHR Biomedical Research Centre.

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Authors and Affiliations

  1. Department of General Surgery, University of Manchester and Central Manchester University Hospitals NHS Foundation Trust, Manchester Royal Infirmary, Oxford Road, Manchester, M13 9WL, UK

    Emma Barrow & James Hill

  2. Genetic Medicine, The University of Manchester, Manchester Academic Health Science Centre, Central Manchester University Hospitals NHS Foundation Trust, St Mary’s Hospital, Oxford Road, Manchester, M13 9WL, UK

    D. Gareth Evans

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  1. Emma Barrow
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  2. James Hill
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  3. D. Gareth Evans
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Correspondence to Emma Barrow.

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Barrow, E., Hill, J. & Evans, D.G. Cancer risk in Lynch Syndrome. Familial Cancer 12, 229–240 (2013). https://doi.org/10.1007/s10689-013-9615-1

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