Introduction: Angiosarcoma of bone is an uncommon primary bone neoplasm that is composed of tumor cells that show endothelial differentiation. This is an aggressive malignancy characterized by frequent local recurrence and distant metastases. The majority of patients die within one year of diagnosis, and this shows that angiosarcoma of bone is an aggressive high-grade tumor.
Case presentation: We present the case of a 65-year-old Japanese woman who had primary angiosarcoma of the proximal humerus with a pathological fracture. An open biopsy confirmed a diagnosis of primary angiosarcoma of bone. Our patient was treated with neoadjuvant chemotherapy and wide resection. One month after surgery, she developed multifocal distant metastasis to her liver and spleen.
Conclusions: Angiosarcoma of the humerus is extremely rare. Radiographically, there is no specific finding associated with angiosarcoma of bone as opposed to other malignant bone tumors. The cornerstone of treatment is en bloc resection followed by as much adjuvant radiation therapy as possible. However, the role of chemotherapy remains undefined, and better systemic agents are clearly needed.