In marked contrast to benign cardiac tumors, primary cardiac sarcomas occur infrequently. Moreover, there is no uniform approach to treating such patients, and the benefits of postoperative chemotherapy are unclear. Between 1964 and 1989, 21 patients with primary cardiac sarcomas underwent surgical resection alone (n = 7), chemotherapy alone (n = 1), or combined operation and postoperative chemotherapy based on adriamycin (n = 13). Twenty-four operations were performed on 20 patients with relief of symptoms in all. Eleven patients had complete resection. Operative mortality was 8.3% (2/24). Histology and originating chamber(s) included angiosarcoma (n = 7; 6/7 in right atrium, 1 in left atrium), malignant fibrous histocytoma (7; all in left atrium), fibrosarcoma (2; 2/2 in left atrium), rhabdomyosarcoma (2; 1 in left atrium, 1 in right ventricle), leiomyosarcoma (2; 1 in left atrium, 1 in left ventricle); and one undifferentiated sarcoma (right atrium). Overall actuarial survival was 14% at 24 months after resection. Patients with complete resection had a median survival of 24 months compared with only 10 months in all other patients (p = 0.035). Postoperative chemotherapy did not enhance survival in patients with incomplete resection. At this time, aggressive and complete surgical resection seems to offer the best hope for palliation and survival in an otherwise fatal disease.