Systemic management strategies for metastatic soft tissue sarcoma.

Soft tissue sarcomas are rare tumours in adults and therefore require a multidisciplinary approach for optimal management. In the metastatic setting, chemotherapy is the primary modality of therapy. Doxorubicin alone or in combination with ifosfamide or dacarbazine has been the backbone of therapy since the 1970s. There is considerable activity for gemcitabine and docetaxel in leiomyosarcoma and for paclitaxel in angiosarcoma. Newer agents such as trabectedin and eribulin may have a role in certain sarcoma subtypes. Palifosfamide may offer a safer alternative to ifosfamide in the future. Many sarcomas have molecular aberrations that can be targeted. Agents that inhibit the insulin-like growth factor receptor-1, mammalian target of rapamycin and vascular endothelial growth factor are currently being investigated.