Their lifetime cumulative risk of endometrial cancer is 40–60 % [26, 28, 29, 31] and that of ovarian cancer is 10–12 % [18, 21, 27, 32–34], appearing to be particularly high for MSH2-mutation carriers [35–37] and accounting for 2 % of all ovarian cancers [34]. The gene discussed is MSH2; the disease is ovarian cancer.