Revised guidelines for the clinical management of Lynch syndrome (HNPCC): recommendations by a group of European experts

Lynch syndrome (LS) (previously referred to as hereditary non-polyposis colorectal cancer; HNPCC) is an autosomal dominant condition caused by a defect in one of the mismatch repair (MMR) genes.1 The syndrome is characterised by the development of colorectal cancer (CRC), endometrial cancer (EC) and various other cancers frequently diagnosed at an early age. LS is probably the most common hereditary CRC syndrome accounting for approximately 1–3% of all CRC. It has been estimated that in Europe approximately one million individuals are carriers of an MMR defect.2

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