Nonetheless, the inhibition of PDGFRα with olaratumab (a human IgG1 antibody targeting PDGFRα) did not showed an enhancement of chemotherapy activity in advanced STS; the phase 3 clinical trial testing the combination of olaratumab and doxorubicin versus doxorubicin did not meet its primary endpoint [7]. This evidence concerns the gene PDGFRA and telomere syndrome.