Upper Gastrointestinal Cancer Surveillance in Lynch Syndrome

SIMPLE SUMMARY: Lynch syndrome is the most common cause of hereditary colorectal cancer, but is also associated with increased extracolonic cancer risk, including upper gastrointestinal cancers. While there is agreement regarding the benefit of frequent colonoscopic surveillance in Lynch syndrome, there remains a lack of consensus on the use of upper gastrointestinal cancer surveillance. Here, we review the upper gastrointestinal cancer risks in Lynch syndrome, the varying guideline recommendations in this area, and the published outcomes of upper gastrointestinal cancer surveillance in this high-risk population. Finally, we highlight ongoing controversies in upper gastrointestinal cancer surveillance and opine on how upper gastrointestinal cancer surveillance can be incorporated into a Lynch syndrome risk management program. Upper gastrointestinal cancer surveillance is an increasingly studied area of risk management in Lynch syndrome, and continued research will be vital in determining how to best incorporate this surveillance in these high-risk patients. ABSTRACT: Lynch syndrome is a common hereditary cancer predisposition syndrome associated with increased digestive cancer risk including colorectal, gastric, and duodenal cancers. While colorectal cancer surveillance is widely accepted to be an important part of a comprehensive Lynch syndrome risk management plan, the use of upper gastrointestinal cancer surveillance in Lynch syndrome remains more controversial. Currently, upper gastrointestinal cancer surveillance guidelines for Lynch syndrome vary widely, and there is no consensus on who should undergo upper gastrointestinal cancer surveillance, how surveillance should be performed, the age at which to initiate surveillance, or how often individuals with Lynch syndrome should undergo upper gastrointestinal cancer surveillance. Fortunately, research groups around the world have been focusing on upper gastrointestinal cancer surveillance in Lynch syndrome, and recent evidence in this field has demonstrated that upper gastrointestinal cancer surveillance can be performed with identification of precancerous lesions as well as early-stage upper gastrointestinal cancers. In this manuscript, we review the upper gastrointestinal cancer risks in Lynch syndrome, differing guideline recommendations for surveillance, outcomes of upper gastrointestinal cancer surveillance, and controversies in the field, and we provide a framework based on our collective experience with which to incorporate upper gastrointestinal cancer surveillance into a risk management program for individuals with Lynch syndrome.