Indication and Extent of Surgery in Hereditary Nonpolyposis Colorectal Cancer

The extent of surgical resection for patients with Lynch syndrome (ie, hereditary nonpolyposis colorectal cancer) remains controversial. More extensive than standard segmental resections reduce the risk of metachronous cancers and simplify surveillance, but a survival benefit has not been documented. The fact that even in hereditary nonpolyposis colorectal cancer these colorectal cancers follow an “adenoma to carcinoma” sequence, albeit accelerated, have brought to question the need for prophylactic risk-reducing surgery of normal bowel segments in addition to the cancer-bearing segment. It has been advocated that adequate endoscopic surveillance may curtail the development of malignancy by treating adenomas as they develop. The high risk of gynecologic malignancy in women adds an additional level of complexity to the surgical decision making and the need and timing of surgery in this subset of patients is in debate. This article reviews the literature to date regarding the extent of surgical resection, and addresses the approach to prophylactic measures to both colonic and gynecologic aspects of malignancy in hereditary nonpolyposis colorectal cancer.