Hereditary non-polyposis colorectal cancer (HNPCC); Lynch syndrome (mutations in DNA mismatch repair (MMR) genes, MLH1, MSH2, PMS1, PMS2, MSH6 and EPCAM) [20,21,22,23,24]; familial adenomatous polyposis coli (FAP) (specific germline mutations in APC genes–constitutive activation of Wnt pathway), Cowden syndrome, among others have been implicated in the latter [14, 20, 25, 26]. Here, MSH6 is linked to Cowden disease.