Primary malignant hemangioendothelioma of bone, also known as angiosarcoma or vascular endothelial sarcoma, is extremely rare. It was first reported by Kokodny in 1926. By 1971, there were only a hundred cases in foreign literature, and few reports in China. The definition proposed by Huvos refers to "tumor cells with anaplastic immature manifestations that form irregular but mutually anastomosing lumens in the tumor, with one or several layers of atypical endothelial cells as the border". Spjut proposed: "Hemangioendothelial sarcoma is a rare malignant tumor that originates from the vascular system cells of the bone or its precursor cells. The endothelial cells have obvious tumor cell manifestations and tend to form curved and mutually anastomosing blood vessels". This disease accounts for 0.1% to 0.4% of bone tumors, 0.5% to 1% of malignant bone tumors, and 4.2% of primary spinal tumors. It is more common in men than in women, and 70% of those aged 10 to 40 years old are affected. This disease is mostly single, and occasionally multiple. The most common site is the long bones of the limbs. The spine is rare, mainly located in the thoracic, lumbar and sacral vertebrae, with occasional case reports of the cervical spine. The tumor is composed of bone vascular endothelial cells or mesenchymal cells that differentiate into endothelial cells. It is highly malignant, grows rapidly, and often metastasizes to the lungs at an early stage.