While monoallelic germline MSH3 pathogenic variants do not cause Lynch syndrome, again possibly because of the stability of the wild-type allele, biallelic MSH3 pathogenic variants present clinically not with the typical CMMRD phenotype involving multiple organs, but just as adenomatous polyposis characterized by EMAST (elevated microsatellite alterations at selected tetranucleotide repeats) in the polyps [13]. The gene discussed is MSH3; the disease is Lynch syndrome.