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Surgical treatment of primary cardiac sarcomas.

Primary cardiac tumors are rare: the autopsy incidence is 0.0001% to 0.0003%, or, in practical terms, about 1 in every 500 cardiac surgical cases. These primary cardiac tumors are 75% benign and 25% malignant, and, of the malignant tumors, 75% are sarcomas.1 Primary cardiac sarcomas often afflict young patients who have no predisposing factors, and these tend to have a dismal prognosis. In studies involving medical therapy alone, 90% of primary-cardiac-sarcoma patients have been dead within 9 to 12 months. Series of soft-tissue sarcomas outside of the heart have shown that complete surgical excision—or sequential resection of metastatic disease, when possible—yields the best long-term survival. 
 
Surgical series of primary cardiac sarcoma have tended to be small, and individual surgeons (and even institutions) have not gained extensive experience with this condition. The Mayo Clinic2 reviewed its records covering a 32-year period and found 34 patients who had undergone surgical resection of primary cardiac sarcoma; the median survival time of 12 months was extended to 17 months in the event of R0 resection. Patients in this series who had an R1 resection had a median survival time of 6 months. A combined series from M.D. Anderson Cancer Center and the Texas Heart Institute3 found 21 patients over a 25-year period; those patients had an actuarial survival rate of 14% at 2 years. Those who underwent an R0 resection had a median survival time of 24 months, whereas those who underwent an R1 resection had a median survival time of 10 months, which again emphasizes the importance of complete resection in these patients. 
 
This background information from the medical literature led our group to a systematic study of primary cardiac sarcoma, in an attempt to better define this difficult disease and to improve the poor prognosis associated with it. We classify primary cardiac sarcoma by location rather than by cell type, because we have found that cell type does not affect prognosis or treatment planning—but anatomic location does. We divide these tumors into right-heart sarcomas, left-heart sarcomas, and pulmonary artery sarcomas.4,5