Meeting ReportsNew clinical criteria for hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the International Collaborative Group on HNPCC☆
Section snippets
Definition of HNPCC
HNPCC is an autosomal dominantly inherited disorder of cancer susceptibility with high penetrance (80%-85%).7, 8, 9, 10, 11 To date the only known cause is an inherited mutation in one of the following mismatch repair (MMR) genes: hMSH2, hMLH1, PMS1, PMS2, and hMSH6.12, 13, 14 The disorder is characterized by the development of colorectal cancer that is often diagnosed at an early age (mean age, 45 years), is multiple (synchronous or metachronous colorectal cancer in 35% of patients), is
New ICG research criteria that include extracolonic cancers
Extensive discussions within the ICG during the last 2 years have led to the following considerations regarding the establishment of additional criteria. First, such criteria should be simple and should not diverge too greatly from the classical criteria. The criteria should be clinical because genetic analyses (MSI analysis, mutation analysis) are not accessible to all families, the techniques are not available in all countries, and the significance of the findings is not completely
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Address requests for reprints to: H. F. A. Vasen, M.D., Ph.D., The Netherlands Foundation for the Detection of Hereditary Tumours, c/o University Hospital, Rijnsburgerweg 10, “Poortgebouw Zuid,” 2333 AA Leiden, The Netherlands. e-mail: [email protected]; fax: (31) 71-5212137.