Hereditary colorectal carcinomas - Reflection on preventive surgery

Hereditary Nonpolyposis Colorectal Cancer (HNPCC) accounts for about 5% of all colorectal cancers and is the most frequent familial form; familial ade nomatous polyposis coli accounts for about 1%. Prerequisitive for individua lly tailored surveillance is the identification of the pathogenic germline mutation. In classical FAP, surgical standard is a restorative proctocolect omy while in HNPCC there is no surgical standard other than standard oncolo gical resection due to missing evidence. In HNPCC, prophylactic colectomy b efore the onset of the first colorectal cancer is not recommended. Main arg uments for the extension of the resection in the case of the first colorect al carcinoma in HNPCC are the rate of metachronous colorectal carcinomas of 40-45% in a 10-year interval and rapid tumor progression. In HNPCC, in the case of first colon cancer a subtotal colectomy seems to be indicated. A p roctocolectomy or, if indicated, a restorative proctocolectomy may be consi dered in the case of carcinomas in the lower rectum. These considerations s hould be evaluated in a prospective clinical trial. Counselling, molecular diagnosis and surgery in patients with hereditary colorectal cancers should only be performed in interdisciplinary centers.