[A case of cardiac angiosarcoma successfully treated with docetaxel]

We report a case of angiosarcoma of the right atrium presenting superior vena cava syndrome. The patient was a 61-year-old man. Echocardiography, CT and MRI revealed a tumor arising in the anterior wall of the right atrium. The tumor was hen-egg sized and unresectable because of the invasion of the pericardium, the right ventricular wall and the superior vena cava. An open biopsy and left brachiocephalic vein-right atrium bypass grafting were performed. The pathological diagnosis was angiosarcoma. The patient agreed to chemotherapy with docetaxel, which is known to be often effective against angiosarcoma of the scalp or face. After 5 courses of docetaxel administration (30 mg/m2 on day 1, 8 and 15 followed by 14 days. rest as one course), echocardiography and CT showed a remarkable tumor reduction, which was evaluated as a partial response. The chemotherapy was suspended for 8 months because of neutropenia and general fatigue as side effects of docetaxel. The administration of docetaxel was resumed and 4 courses were performed. The tumor, however, became resistant to docetaxel and formed metastatic involvements in the liver. Following treatments with paclitaxel, IL-2 and CPT-11 were ineffective for the primary tumor and liver metastases. He died of cardiac tamponade caused by massive hemorrhage into the pericardiac space from the tumor surface. He had long-term survival 31 months after the diagnosis. An effective treatment for cardiac angiosarcoma has not yet been established. Chemotherapy with docetaxel should be considered in the treatment of patients with cardiac angiosarcoma.