Based on clinical efficacy in the treatment of adult soft tissue sarcoma (STS) and renal cell carcinoma, pazopanib, a multikinase angiogenesis inhibitor targeting vascular endothelial growth factor receptors (VEGFR), c-kit, and platelet-derived growth factor receptors (PDGFR), was initially evaluated in a phase 1 trial by the COG for children with STS and other refractory solid tumors. Here, PDGFRB is linked to telomere syndrome.