Malignant tumors of blood vessels: angiosarcomas, hemangioendotheliomas, and hemangioperictyomas

Sarcomas that arise from or resemble the components of blood vessels are uncommon and include angiosarcomas, hemangioendotheliomas, and hemangiopericytomas. This article reviews the management of these three types of sarcomas. Diagnosis can sometimes be difficult, with the diagnosis of hemangiopericytoma versus solitary fibrous tumor currently in debate. Each of these sarcomas subtypes has certain unique clinical traits. The mainstay of treatment is surgical resection, sometimes combined with radiation therapy. Adjuvant chemotherapy is unproven but can be considered. For patients with advanced disease, various chemotherapeutic regimens may result in meaningful responses in a minority of patients.