Olaratumab (Lartruvo®) is a 154 kDa fully recombinant human IgG1 monoclonal antibody with high affinity to PDGFRα, which is the first-line drug approved by FDA for soft tissue sarcoma (STS) (Table 1).385 STS is a relatively rare malignancy that occurs in connective tissue. Here, PDGFRA is linked to telomere syndrome.