Surgical management of hereditary nonpolyposis colorectal cancer

HNPCC is a diverse disease with significant colorectal and extracolonic malignancy risk. A high index of suspicion is necessary to identify patients and families who potentially have this disease. Patients suspected with Lynch syndrome should be referred for genetic counseling and testing for accurate diagnosis. Timely surveillance and intervention are essential to reduce the incidence and mortality from colorectal cancer. Once cancer is diagnosed, aggressive surgical management is warranted because there is significant metachronous colorectal neoplasia risk for all remaining colorectal mucosa. In medically fit patients, consideration should be given to colectomy for the treatment of colon cancer and proctocolectomy for the treatment of rectal cancer. For patients treated with anything less than total proctocolectomy, annual endoscopic surveillance of the remaining colorectum is mandatory.