Hemangiosarcoma is a soft tissue sarcoma derived from vascular or lymphatic endothelial cells, accounting for 1%-2% of soft tissue sarcomas and 5.4% of soft tissue sarcomas of the skin. They are highly malignant, highly invasive and have poor prognosis. They can occur in any site, most commonly in the head and neck skin of elderly white men. Radiotherapy or chronic lymphedema was highly associated with secondary hemangiosarcoma. The pathological diagnosis was the gold standard. Microscopically, extensive local or extensive solid infiltration of spindle cells in the dermis was observed, and cell atypia was common. Immunohistochemistry was usually positive for CD31, CD34 and Ⅷ factor. Radical surgery is the preferred treatment. For patients with high risk of local recurrence, large dose (>50 Gy) large range adjuvant radiotherapy is recommended. Further radiotherapy should be avoided for hemangiosarcoma secondary to radiotherapy. Chemotherapy is the primary treatment for advanced and metastatic angiosarcoma. Targeted therapy and immunotherapy have developed rapidly in recent years. Isolated limb perfusion (ILP) can be used as an alternative to radical surgery.

Angiosarcoma; Pathogenesis; Diagnosis; Comprehensive treatment