Surgery, chemotherapy, and radiation therapy

Multimodal treatment with surgery, systemic chemotherapy, and radiation therapy is used for metastatic disease, although it is rarely curative.[33,34] Disease control is the objective in patients with metastatic angiosarcomas. Published progression-free survival is between 3 months and 7 months,[35] and the median overall survival (OS) is 14 to 18 months.[36] In both adults and children, the 5-year OS rates are between 20% and 35%.[21,22,37]

One child who was diagnosed with angiosarcoma secondary to malignant transformation from infantile hemangioma responded to treatment with bevacizumab (a monoclonal antibody against vascular endothelial growth factor) combined with systemic chemotherapy.[19,34]

Biologic agents that inhibit angiogenesis have shown activity in adults with angiosarcomas.[20,37]

There is one case report of a pediatric patient with metastatic cardiac angiosarcoma who was successfully treated with conventional chemotherapy, radiation, surgery, and targeted therapies, including pazopanib.[38]