This latter is caused by pathogenic germline variants in Mismatch Repair Genes, MLH1, MSH2, MSH6 or PMS2, and predispose mainly to colorectal cancer and to other malignancies including endometrial, gastrointestinal, ovarian, pancreatic and skin cancers (Pande et al., 2012). The gene discussed is MSH6; the disease is skin neoplasm.