The cumulative risk for urinary tract cancer in LS patients varies, ranging from less than 1% to 28%, with greater risk among carriers of MSH2 mutations (2–28%) compared to carriers of MLH1 (0.2–5%) or MSH6 (0.7–5.5%) mutations [14,15,21,22,23,24,25,26]. This evidence concerns the gene MSH2 and Leigh syndrome.