Remarkably, the concordance of germline pathogenic variants and loss of protein expression or presence of MSI was higher for the MSH2 gene, since 87% (21/24) of sarcomas from MSH2 carriers harbored one of these hallmarks, in contrast to 75% for MLH1 (6/8) and MSH6 (3/4) and 0% for PSM2 (0/1) (Table 3). Here, MSH2 is linked to sarcoma.