Management of angiosarcoma

Angiosarcoma is an uncommon tumor that presents in several scenarios. Although angiosarcoma can occur in any organ, it typically presents in the following settings: 1) in the face or scalp among elderly patients, 2) with chronic lymphedema, 3) after radiotherapy, or 4) as a hepatic primary. In all of these situations, angiosarcoma is associated with multifocality and an insidious growth pattern, making local control difficult. Doxorubicin-ifosfamide chemotherapy produces a modest response rate, and paclitaxel appears to be useful for scalp and facial angiosarcomas. However, improvement in systemic therapy is clearly needed. One logical strategy is to investigate antiangiogenic therapies in this endothelial tumor. Improved therapy for this tumor will come only with greater understanding of its biology.