The Lynch syndrome (HNPCC) is characterized by the development of colorectal cancer, endometrial cancer and various other cancers and is caused by a mutation in one of the mismatch repair (MMR) genes: MLH1, MSH2, MSH6 or PMS2.The discovery of these genes 15 years ago, has led to the identification of large numbers of affected families. In April 2006, a workshop was organized by a group of European experts in Hereditary Gastrointestinal Cancer (the Mallorca-group) aiming to establish guidelines for the clinical management of Lynch syndrome. Twenty-one experts from nine European countries participated in this workshop. Prior to the meeting, various participants prepared the key management issues of debate according to the latest publications. A systematic literature search using Pubmed and the Cochrane Database of Systematic Reviews, reference lists of retrieved articles, and manual searches of relevant articles was performed. During the workshop all recommendations were discussed in detail. Because most of the studies that form the basis for the recommendations were descriptive and/or retrospective of nature, many of them were based on expert opinion. The guidelines described in this manuscript may be helpful to the appropriate management of Lynch syndrome families. In order to further improve the care of these families, prospective controlled studies should be undertaken.
- Lynch syndrome
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