Novel treatment targets in sarcoma: more than just the GIST

Sarcomas are rare tumors comprising a heterogeneous group of more than 50 histologic subtypes, the majority of which do not respond well to cytotoxic chemotherapy. This has fueled research into the distinct molecular mechanisms of tumorigenesis and disease progression for various sarcoma subtypes. Gastrointestinal stromal tumors and liposarcomas are presented as paradigms of molecular classification that have led to the rational development of novel therapeutic strategies for those tumors. Recent advances in understanding of growth signaling pathways, metabolic reprogramming, and immune therapy have identified new treatment targets for many sarcomas. These investigations will form the foundation for further improvements in our ability to care for patients with these tumors and may offer clinical insights into a wide range of other tumors.