Abstract
Soft tissue sarcoma is a malignancy that develops from human soft tissues such as muscle, nerve, fat, and blood vessels. The World Health Organization classification comprises about 50 different histologic types of soft tissue sarcoma. Soft tissue sarcoma is treated most often with surgery. Chemotherapy and radiotherapy have shown only minor effects on patient survival in this disease. The overall 5-year survival rate of soft tissue sarcoma is 50%; it has not changed for the past several decades. A new class of therapeutic targets for soft tissue sarcoma was identified recently. Epigenetic regulators, such as DNA methyltransferases, histone deacetylases, and histone-modifying enzyme enhancer of zeste homolog 2, have been found to be involved in pathogenesis of various soft tissue sarcomas. Small-molecule inhibitors of these epigenetic regulators may provide a new targeted therapy approach to soft tissue sarcomas in the future.