Angiosarcoma is a highly malignancy of endothelial tumor and represents 1-2% of all soft tissue sarcomas in humans. The aetiology of angiosarcoma is not clear but there are definite risk factors including chronic lymphoedema, history of radiation, environmental carcinogens and certain familial syndromes. Ultrasound, CT and MR are diagnostic tools, but final diagnosis requires pathological and immunohistochemical confirmation. The conventional options of treatment include surgery, radiotherapy and chemotherapy. Targeted medicines and immunotherapy have been studied as promising treatment of angiosarcoma. The goal of this review is to summarize the current data regarding of angiosarcoma and its clinical presentation and management, providing a useful clinical tool to explore the optimal treatment.
Keywords: Angiosarcoma; aetiology; chemotherapy; diagnosis; prognostic factors; targeted therapy.
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