Angiosarcoma

Excerpt

Angiosarcoma (AS) comprises 1% of all soft-tissue sarcoma (STS), which are themselves a rare malignancy. They arise from lymphatic or vascular endothelial cells and are 'high-grade' by definition, which demonstrates their aggressive behavior. Although AS can occur in any part of the body, it most commonly presents as a cutaneous disease in elderly white men or on the chest wall after receiving radiation therapy (RT) for breast cancer. The treatment is very challenging, and the prognosis is poor, especially if AS is diagnosed in the metastatic stage. The best approach to patients with AS is offered in a multidisciplinary tumor board setting. Like any other STS, surgical resection with a negative margin affords the best outcomes in terms of overall survival. Combining radiation therapy (RT) with weekly paclitaxel has been demonstrated to bear durable responses for cutaneous angiosarcoma. Doxorubicin and paclitaxel are recommended regimens for advanced or metastatic disease. Due to the increased vascularity of AS, targeted therapy against vascular endothelial growth factor (VEGF) has gained traction, however, yet remains to be proven in a prospective study.