Role of chemotherapy in the management of soft tissue sarcomas.

Soft tissue sarcomas are a diverse group of rare tumors that comprise 1% of all cancers. Few randomized trials of chemotherapy have been performed but there is a clear role for agents such as doxorubicin and ifosfamide in the palliation of advanced disease. There is uncertainty as to whether sequential single-agent treatment is equivalent to combination chemotherapy. For the majority of histological subtypes adjuvant chemotherapy is not of proven value, although there may be situations where it is advantageous. However, there are other subtypes, such as the Ewing's sarcoma family tumors, for which chemotherapy is an essential part of primary management and has definitely improved survival. Apart from Ewing's sarcoma family tumor and rhabdomyosarcoma, there is increasing specialization of chemotherapy according to histological subtype, such as the use of taxanes for angiosarcoma, gemcitabine and docetaxel for leiomyosarcoma, and trabectedin for leiomyosarcoma and liposarcoma, especially the myxoid/round cell variant. Nevertheless, there are serious limitations to existing treatment and novel therapies need to be developed.