A 45-year-old black patient presented with right-heart failure. Echocardiography showed a large tumor mass in the right atrium and a debulking procedure was performed. Histological and immunohistochemical studies revealed a high grade angiosarcoma infiltrating the myocardium and extending to the pericardial surface. Metastatic work-up showed no evidence of dissemination. The patient made an uneventful postsurgical recovery and was treated with chemotherapy, consisting of epidoxorubicin and dacarbazin. Following chemotherapy, echocardiographic re-evaluation failed to show any residual tumor in the right atrium. At present, one year following diagnosis, the patient is alive with no evidence of disease. Therapeutic options of primary cardiac angiosarcoma are reviewed.
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