Indeed, nearly all CRC and most extracolonic tumors from LS patients are MSI/dMMR, but only 22% of MSI/dMMR CRC [17,43] and 8–14% of MSI/dMMR endometrial cancers [20,43,45] harbor MMR germline mutations, requiring additional tools to identify LS (Table 2). This evidence concerns the gene MRC1 and colorectal carcinoma.