Angiosarcoma is a malignant tumor originated in the vascular or lymphatic endothelium. As described in the literature under various names, angiosarcomas were previously called vascular endothelial tumor, ductal sarcoma, angiosarcoma, malignant hemangioendothelioma, and lymphatic sarcoma. It is now known as angiosarcoma, including vascular endothelial derived hemangiosarcoma and lymphangiosarcoma. Hemangiosarcoma cannot be differentiated from lymphangiosarcoma due to lack of reliable morphologic parameters and immunohistochemical features, so they are collectively referred to as angiosarcoma. Angiosarcoma is a rare tumor, commonly seen in skin and soft tissue, accounting for approximately 1–2% of soft tissue sarcoma. Retroperitoneal angiosarcoma is extremely rare as reported in the literature. In this chapter, etiological causes, histopathological features, immunohistochemical markers, clinical manifestation, treatment strategies, therapeutic efficacy, and prognostic factors of angiosarcoma will be briefly described.
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