Soft tissue sarcomas (STS) represent a heterogeneous group of diverse neoplasms of mesenchymal origin. Once relapsed from standard therapy, STS patients have limited treatment options especially those that present with advanced or metastatic disease. In this review article, we highlight recent clinical data that led to the US Food and Drug Administration (FDA) approval of pazopanib (Votrient®) for STS and regorafenib (Stivarga®, BAY 73-4506) in gastrointestinal stromal tumours. We also review ongoing safety/efficacy data for trabectedin (Yondelis®, ET-743), and data from clinical studies of ridaforolimus (AP23573; MK-8669) and palifosfamide (ZIO-201). We provide a list of some promising ongoing trials in soft tissue sarcomas including first line studies of TH-302 and trabectedin. Finally, our article delves into recent advances in our understanding of the molecular pathogenesis of STS and novel therapies that might be explored as treatment options for specific STS histologies.
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