Familial Cancer

, Volume 12, Issue 2, pp 229–240

Cancer risk in Lynch Syndrome

Original Article

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.

Keywords

Lynch Syndrome Hereditary nonpolyposis colorectal cancer (HNPCC) DNA mismatch repair Familial relative risk Risk reduction Statistical bias 

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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Copyright information

© Springer Science+Business Media Dordrecht 2013

Authors and Affiliations

  1. 1.Department of General SurgeryUniversity of Manchester and Central Manchester University Hospitals NHS Foundation Trust, Manchester Royal InfirmaryManchesterUK
  2. 2.Genetic MedicineThe University of Manchester, Manchester Academic Health Science Centre, Central Manchester University Hospitals NHS Foundation Trust, St Mary’s HospitalManchesterUK

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