Hereditary non-polyposis colorectal cancer.

Hereditary non-polyposis colorectal cancer (HNPCC) is an autosomal dominant condition in which affected individuals develop colorectal cancer or extra colonic cancer, most commonly endometrial, at an early age. Recent advances in molecular genetics have led to the identification of a germline mutatio n of DNA mismatch-repair genes to be responsible for the majority of HNPPC cases. Since clinical screeening of gene carriers can help to prevent cance r, it is important to devise strategies applicable to this syndrome. Recomm endations for current management, especially screeening and surgical treatm ent are under study, as they have not yet been clearly established. This pa per reviews the: clinical presentation, the molecular genetic diagnosis and therapeutic approaches of this syndrome including the controversies concer ning prophylactic colectomy for cancer or gene carriers.