Analysis of the age of cancer onset stratified by each altered MMR gene revealed that the average age of CRC onset was 42.7 years (range 29–63) in the 12 MLH1 PV/LPV carriers, 38.1 years (range 32–54) in the 8 patients harboring MSH2 PVs, and 60.8 years (range 45–69) in the 6 patients harboring MSH6 alterations. Here, MSH2 is linked to colorectal carcinoma.