Prophylactic surgery for hereditary nonpolyposis colorectal cancer

The most frequent hereditary colorectal cancer predisposition is Lynch syndrome, or hereditary nonpolyposis colorectal cancer. The option of prophylactic surgery relies on the penetrance of the genetic defect and the heterogeneity of the condition. Since 20% of all mutation carriers never move on to develop cancer, the purely prophylactic setting is not indicated. However, when colorectal cancer is diagnosed, the question arises if the patient may benefit from extended surgery - total colectomy or (restorative) proctocolectomy. These patients should be entered into the ongoing prospective-randomized study by German Cancer Aid (http://www.hnpcc-studie.de). Due to substantially increased cancer risk and poor surveillance options, the endometrium and stomach are also subject to the question of prophylactic intervention.