Primary cardiac sarcoma.

BACKGROUND: The presentation, management, and outcome of patients with primary cardiac sarcomas are not well defined. Furthermore, the role of adjuvant therapy has not been delineated in the management of primary cardiac sarcomas.

METHODS: Patients with primary cardiac sarcoma and noncardiac sarcoma, diagnosed between 1988 and 2005, were identified in the Surveillance, Epidemiology, and End Results (SEER) database. Clinical characteristics and outcomes of primary cardiac sarcoma were defined and compared with the characteristics of noncardiac sarcomas. Univariate and multivariate methods were used to identify factors associated with primary cardiac sarcoma survival.

RESULTS: Compared with noncardiac sarcomas, primary cardiac sarcomas were found to occur in a younger age group and were more likely to present with advanced disease. Primary cardiac sarcomas were ten times more likely to be vessel-derived (eg, angiosarcoma), comprising almost half of all cases. Median overall survival for cardiac sarcoma patients was 6 months whereas that for noncardiac sarcoma patients was significantly longer at 93 months (p < 0.001). Furthermore, cardiac sarcoma patients who underwent surgery had a median survival of 12 months whereas those who did not undergo surgery had a median survival of 1 month (p < 0.001).

CONCLUSIONS: Cardiac sarcomas are a distinct, rare subset of soft tissue sarcomas with a poor prognosis. Surgery continues to be the central component of successful management. Future clinical efforts should be directed at developing approaches to permit safe radical excision and, potentially, developing effective adjuvant therapy.