The risk of developing other extracolonic cancers is also much lower in carriers of a PV in MSH6 or PMS2 compared to MLH1 and MSH2 [5,9,10,11], with one major exception: female carriers of a PV in MSH6 have high life-time risks for gynecological cancers (41% for EC, up to 11% for ovarian cancer), comparable to carriers of a PV in MLH1 or MSH2 [8]. Here, MLH1 is linked to ovarian carcinoma.