Soft tissue sarcomas are a heterogeneous group of rare malignancies with few effective standard therapies. Our understanding of the underlying biology driving tumorigenesis in these mesenchymal tumors have led to a growth in drug development for soft tissue sarcomas. This review focuses on novel targets in soft tissue sarcomas, describes early clinical trial results of drugs directed at these targets, and discusses the data surrounding the use of these compounds in clinical practice and rationale for possible future US Food and Drug Administration approvals.

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Competing Interests

Source of Support: None. Conflict of Interest: Shiraj Sen receives research funding for clinical trials (paid in full to his institution) from LOXO, Jacobio, Exelixis, GSK, BioAtla, Xencor, Epizyme, Abbisko, Fujifilm, Synthorx, Turning Point Therapeutics, Daiichi Sankyo, Tesaro. The other authors have nothing to disclose.