Surgery of hereditary colorectal cancer

Hereditary colorectal cancer syndromes account for about 7% of all colorect al carcinomas. The most frequent form is Hereditary Nonpolyposis Colorectal Cancer (HNPCC). Identification, cloning and sequence analysis of the predi sposing genes enables identification of mutation carriers and non-mutation carriers, respectively. These genetic informations can be used in an indivi dually tailored clinical surveillance program and may ultimately result in standard preventive surgical treatment. In classical FAP the surgical stand ard is performing a restaurative proctocolectomy. It is still unclear now, if this procedure should be modified in attenuated forms (colectomy with il eorectostomy). Due to a high rate of synchronous and metachronous carcinomas a subtotal co lectomy in the case of first colon cancer seems to be indicated in HNPCC pa tients. A proctocolectomy or a restaurative proctocolectomy should be weigh ed in case of carcinomas in the lower rectum. These procedures should be pe rformed under the precondition of identification of the pathogenic germline mutation in the patient, only. In addition, a synchronous prophylactic hys terectomy with oophorectomy should be recommended postmenopausal gene carri ers. Intensive counseling of the patient should preceed these preventive pr ocedures involving surgeons, gastroenterologists, geneticists, molecular bi ologists, gynecologists, physicians and psychologists. It is recommended to have patients treated exclusively in specialized centers. Currently, six i nterdisciplinary centers for cancer surveillance and early diagnosis in her editary colorectal cancer are being sponsored in Germany by the Deutsche Kr ebshilfe since 1999. In the future clinical studies have to be conducted to evaluate the efficacy of extended colorectal resections versus efficacy of surveillance and conventional resections according to general oncological principles.