Identifying patients with Lynch syndrome is clinically important because these patients have up to 80 percent lifetime risk of colorectal cancer and up to 60 percent lifetime risk of endometrial cancer.
These patients also have an increased risk for other primary cancers including gastric, ovarian, small bowel, urothelial (ureter, renal pelvis), biliary tract, pancreatic, brain (glioblastoma), sebaceous gland adenomas, and keratoacanthomas.
Individuals found to have a deleterious Lynch syndrome mutation are at increased cancer risk with the greatest risk of colorectal and endometrial cancers, followed by gastric and ovarian cancers. Fortunately, there are risk management guidelines for carriers of Lynch syndrome which are associated with a decrease in cancer-related deaths.