Advances in the systemic treatment of cutaneous sarcomas.

Cutaneous sarcomas are a rare subset of soft tissue sarcomas. These tumors are primarily managed with definitive surgical resection; however, upon unresectable recurrence or metastatic spread, systemic therapy is warranted. As with other sarcomas, these treatments have classically included cytotoxic chemotherapy programs that were associated with variable response rates and poor overall survival. Recently, major advances have been made in the understanding of the molecular biology of these tumors, and treatment paradigms are changing. Multiple pathways have been documented to be important in the growth of cutaneous sarcomas, including receptor tyrosine kinases such as platelet-derived growth factor receptor, insulin-like growth factor receptor and KIT. Dysregulated angiogenesis, through vascular endothelial growth factor (VEGF) and other pathways, is also associated with the growth of these tumors. In this review, we discuss the current standard therapies of cutaneous sarcoma and the recent advances and ongoing investigations into cutaneous sarcoma biology.