Abstract
Hereditary nonpolyposis colorectal cancer (HNPCC) or Lynch syndrome is a disease characterized by autosomal dominant clustering of colorectal cancer (CRC) as well as other cancers. It is critical for clinicians and surgeons caring for patients with HNPCC to be familiar with their management related to CRC. Based on retrospective studies, screening colonoscopy is recommended every 1 to 2 years beginning at age 20 to 25, or 10 years younger than the earliest CRC in the family (whichever is earlier). HNPCC patients with colon cancer should be considered for total abdominal colectomy rather than a more limited segmental colon resection due to the increased risk of metachronous neoplasia associated with the condition. Rectal cancer in HNPCC has not been well studied, but discussions with the patient regarding surgical management should weigh the risks of metachronous CRC with the morbidity and quality of life issues associated with proctocolectomy. Regardless of the procedure, a patient with HNPCC requires close postoperative endoscopic surveillance of any remaining at-risk mucosa. In terms of chemoprevention, aspirin has been shown to be effective in preventing colorectal neoplasia in prospective trials and should be considered in patients who do not have a contraindication to the drug. Trials for other chemopreventative agents in HNPCC are ongoing. As more is learned about particular genotype–phenotype correlations with Lynch syndrome, this will likely affect surgical decision making. Despite all of these efforts in the management of patients with HNPCC or Lynch syndrome, incident CRCs still occur, thus reinforcing the need for further studies to better understand the optimal management of these patients.
Keywords
HNPCC - hereditary nonpolyposis colorectal cancer - Lynch syndrome - colon cancer - colorectal cancer - hereditary cancer syndrome
