syndrome de lynch hnpcc
Lynch syndrome, often called hereditary nonpolyposis colorectal cancer (HNPCC), is an inherited disorder that increases the risk of many types of cancer, particularly cancers of the colon (large intestine) and rectum, which are collectively referred to as colorectal cancer. Typically HNPCC patients present in their forties and fifties with colorectal cancer 2, or with one of the associated malignancies.It is 5 times more common than familial adenomatous polyposis syndromes (FAP) 6.It is the most common hereditary cause of endometrial cancer 9. XII - n°6 - novembre-décembre 2009 DOSSIER THÉMATIQUE Formes héréditaires des cancers colorectaux L e syndrome HNPCC (Hereditary Non Polyposis Colorectal Cancer), ou syndrome de Lynch, est une forme de prédisposition héréditaire aux De plus, l’aspirine pourrait être bénéfique pour d’autres cancers que le CCR. •Identification de nouveaux gènes, parallèlement à l’évolution des techniques de BM •Prise en charge des apparentés difficile •Standardiser-évaluer surveillance des cancers extra-digestifs (endomètre+++) Title: Syndrome HNPCC.ppt Syndrome de Lynch ou HNPCC (Hereditary Non-Polyposis Colorectal Cancer) I. This means people with Lynch syndrome have a higher risk of certain types of cancer. Le syndrome de Lynch est une maladie héréditaire qui donne à une personne un risque augmenté de développer certains cancers de l’appareil digestif, de l’endomètre et d’autres organes. Lynch syndrome is a genetic disorder that causes an increased risk of developing certain types of cancer such as colon and rectal cancer, as well as cancers of the stomach, small intestine, liver, gallbladder ducts, upper urinary tract, brain, skin, and prostate. •HNPCC est un syndrome familial de diagnostic complexe. Hereditary nonpolyposis colorectal cancer (HNPCC) is subdivided into (1) Lynch syndrome I, or site-specific colonic cancer, and (2) Lynch syndrome II, or extracolonic cancer, particularly carcinoma of the stomach, endometrium (see 608089), biliary and pancreatic system, and urinary tract (Lynch and Lynch, 1979; Lynch et al., 1985; Mecklin and Jarvinen, 1991). Lynch syndrome is the most common cancer syndrome, affecting 1 in 400 persons 3.. Le syndrome de Lynch est une maladie génétique augmentant chez un sujet le risque de développer un cancer du côlon, de l’intestin grêle, du foie, de l’estomac et de l’appareil urinaire supérieur. Un diagnostic de syndrome de Lynch devrait être envisagé lorsque plusieurs personnes du même côté de la famille ont reçu un diagnostic de cancer colorectal. (2)Institut für Pathologie, Medizinische Fakultät und Uniklinik Köln, Universität Köln, Kerpener Str. HNPCC was described by Lynch [] in the 1960s and is the cause of around 5% of all CRC.It has been shown that HNPCC is caused by mutations within several genes such as: MSH2, MLH1, MSH6, PMS2.Mutation within MSH2 and MLH1 are the most frequent cause of Lynch syndrome [7–10].Characteristic clinical features of Lynch syndrome include: Au cours du syndrome de Lynch, l’étude CAPP2 a montré que la prise régulière d’aspirine pendant au moins 2 ans entraînait une réduction du risque de CCR de 60 % versus un placebo. Women with Lynch syndrome also have a high risk of developing uterine cancer (also called endometrial cancer) and ovarian cancer. 62, 50937, Köln, Deutschland. Lynch syndrome, also known as hereditary non-polyposis colorectal cancer (HNPCC), is a type of inherited cancer syndrome associated with a genetic predisposition to different cancer types. 210 | e de l’Hépato-gastroentérologue La Lettr • Vol. Epidemiology. Introduction Actuellement, 5 gènes de prédisposition au an er oloretal ont été identifiés, il s’agit des gènes MMR, abréviation de « Mismatch Repair » : MLH1, MSH2, MSH6, PMS2 et EPCAM. nicolaus.friedrichs@uk-koeln.de.