The cumulative lifetime risk for ovarian cancer in LS is reported to be between 7 and 24% up to age 70 years and varies between genotypes, with most older studies reporting the highest risks in MSH2 and MLH1 carriers, as the number of families with pathogenic variants in MSH6 have been too low in most studies for conclusive results [4, 16]. This evidence concerns the gene MLH1 and ovarian cancer.