Revised guidelines for the clinical management of Lynch syndrome (HNPCC): recommendations by a group of European experts
نویسندگان
چکیده
Lynch syndrome (LS) is characterised by the development of colorectal cancer, endometrial cancer and various other cancers, and is caused by a mutation in one of the mismatch repair genes: MLH1, MSH2, MSH6 or PMS2. In 2007, a group of European experts (the Mallorca group) published guidelines for the clinical management of LS. Since then substantial new information has become available necessitating an update of the guidelines. In 2011 and 2012 workshops were organised in Palma de Mallorca. A total of 35 specialists from 13 countries participated in the meetings. The first step was to formulate important clinical questions. Then a systematic literature search was performed using the Pubmed database and manual searches of relevant articles. During the workshops the outcome of the literature search was discussed in detail. The guidelines described in this paper may be helpful for the appropriate management of families with LS. Prospective controlled studies should be undertaken to improve further the care of these families.
منابع مشابه
Hereditary Nonpolyposis Colorectal Cancer (HNPCC)/Lynch Syndrome: Surveillance and Diagnostic strategies
Introduction: Hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) is an autosomal dominant genetic disease. The disease is caused by a mutation in one of four genes of the DNA mismatch repair system and increases the risk for various cancers, especially the uterine and colon cancers. The prevalence of this disease in the general population is about 1 in 500 and it causes about 2-3...
متن کاملGuidelines for the clinical management of Lynch syndrome (hereditary non-polyposis cancer).
Lynch syndrome (hereditary non-polyposis colorectal cancer) is characterised by the development of colorectal cancer, endometrial cancer and various other cancers, and is caused by a mutation in one of the mismatch repair 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...
متن کاملRevised Bethesda Guidelines for hereditary nonpolyposis colorectal cancer (Lynch syndrome) and microsatellite instability.
Hereditary nonpolyposis colorectal cancer (HNPCC), also known as Lynch syndrome, is a common autosomal dominant syndrome characterized by early age at onset, neoplastic lesions, and microsatellite instability (MSI). Because cancers with MSI account for approximately 15% of all colorectal cancers and because of the need for a better understanding of the clinical and histologic manifestations of ...
متن کاملLynch syndrome (HNPCC) and microsatellite instability analysis guidelines.
The “International Workshop on Diagnostic Guidelines for Hereditary Non-Polyposis Colorectal Cancer and Microsatellite Instability” represents the third workshop in this series. In November 1996, NCI’s DCP convened a workshop entitled “Intersection of Pathology and Genetics in HNPCC Syndrome.” In December 1997, another workshop, “International Workshop on Microsatellite Instability and Replicat...
متن کاملسندرم Lynch -l و گزارش شش مورد بیمار مبتلا
More than two decades ago, Henry T. Lynch reported a hereditary non polyposis colorectal cancer (HNPCC) which is seen in some families with dominant mode of inheritance, also know as Lynch Syndrome type I and II. This form to hereditary colorectal cancer has an early age of onset (Less than 45 years) with predominantly proximal colonic involvement (type I) and can also be associated with extrac...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 62 شماره
صفحات -
تاریخ انتشار 2013