A Lynch syndrome prospective study had estimated the cumulative risk of ovarian cancer as a unique disease to be 10% (95% CI 4.8–15.4%) for MLH1 carriers by the age of 75 years, 17% (95% CI 5.7–28.0%) for MSH2 carriers, and 13% (95% CI 0.1–31.2%) for MSH6 carriers [13]. Here, MSH6 is linked to ovarian cancer.