[Systemic therapy of soft tissue sarcomas]

The gold standard for the treatment of primary, resectable, high-grade soft tissue sarcomas is complete surgical removal followed by radiotherapy. In cases where preservation of function is not possible, preoperative treatment options should be considered. Systemic therapy is the treatment of choice for metastatic soft tissue sarcomas. The most active single agents include the anthracyclines doxorubicin and epirubicin, as well as ifosfamide. While combination chemotherapy yields higher response rates, this is at the cost of increased toxicity with no evidence of prolonged overall survival. Current treatment strategies focus on the development of specific treatments for well defined soft tissue sarcoma subtypes. The first and highly successful targeted therapy was seen with the introduction of imatinib in the treatment of gastrointestinal stromal tumors.