Increasing attention has been given to hereditary nonpolyposis colorectal cancer (HNPCC), a disorder which occurs four or five times more frequently than its hereditary counterpart, familial multiple adenomatous polyposis coli (FPC). Because of the lack of premonitory physical signs in HNPCC, its diagnosis must encompass pertinents family cancer history. This report describes a kindred with a subtype of HNPCC, the cancer family syndrome also referred to as Lynch syndrome II. Emphasis has been given to the temporal evolution of this disorder and the manner in which minimal clinical-genetic clues might best be employed for its diagnosis.
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