Abstract
BACKGROUND: Primary angiosarcoma of the breast (PAB) is a rare occurrence of highly aggressive biological behavior. Bilateral PAB is even more infrequent. CASE REPORT: We present the case of a 39-year-old Chinese woman with metachronous bilateral PAB (2005 and 2008). The diagnosis of PAB was confirmed. The respective masses were removed via simple mastectomy. The extent of malignancy differed microscopically between the two tumors and indicated a different source. After two surgeries and a 3-year follow-up, the patient is alive and well. The current case illustrates an unusual presentation of this rare type of breast sarcoma, in that none of the clin-icopathological findings are thought to confer a good prognosis. We also review the literature and summarize relevant findings concerning definition, pathology, clinical features, treatment, and follow-up. CONCLUSIONS: We believe that the survival rate depends on tumor size and differentiation. Surgical resection followed by chemotherapy may prove to be effective and afford the best prognosis in the future.