A recent study by Mas-Moya et al [44] demonstrated that when compared to LS, Lynch-like syndrome is more likely to occur in the right colon (a subset of LS tumors occurs at left colon [45]), more often identified in patients with tumors that harbor concurrent MLH1/PMS2 loss (with lack of MLH1 promoter hypermethylation) or concurrent MSH2/MSH6 loss, and less likely to have synchronous or metachronous LS-associated cancers. This evidence concerns the gene PMS2 and Leigh syndrome.