Familial Cancer

, Volume 12, Issue 2, pp 229–240

Cancer risk in Lynch Syndrome

Original Article

DOI: 10.1007/s10689-013-9615-1

Cite this article as:
Barrow, E., Hill, J. & Evans, D.G. Familial Cancer (2013) 12: 229. doi:10.1007/s10689-013-9615-1


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.


Lynch SyndromeHereditary nonpolyposis colorectal cancer (HNPCC)DNA mismatch repairFamilial relative riskRisk reductionStatistical bias



Hereditary non-polyposis colorectal cancer


Mismatch repair


International Collaborative Group on HNPCC


Colorectal cancer


Central nervous system

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