A study evaluating the risk in 20 MSH6 families showed that colorectal cancer was less frequent and developed 10 years later in MSH6 compared to MLH1 and MSH2. In addition a significant higher lifetime risk of endometrial carcinoma of 71% in MSH6 mutation carriers with a later age of onset (54 years vs. 48 and 49 years for MLH1 and MSH2) was reported [17]. Here, MLH1 is linked to colorectal cancer.