Management of primary cardiac sarcomas.

Cardiac sarcomas are rare entities. The biological behavior of cardiac sarcomas is similar to all soft-tissue sarcomas. Aggressive local growth and metastatic spread are common. Although histologic type affects behavior, survival is dependent on the histologic grade. Chemotherapy and radiation therapy are not adequate for long-term survival. Although surgery provides the best modality for local control it is limited by its inability to control distant metastatic disease. Right heart sarcomas tend to be bulky, infiltrative, cause heart failure late and metastasize early. Based on the surgical approach and clinical behavior, cardiac sarcomas can be classified as right heart sarcomas, left heart sarcomas and pulmonary artery sarcomas. Our limited - albeit the most extensive - experience with cardiac sarcomas has helped improve survival compared with chemotherapy alone. They are usually treated with chemotherapy prior to extensive resection. Left heart sarcomas tend to be more circumscribed, less infiltrative, cause heart failure early and metastasize later. They are usually treated with surgery first, given the possibility of cardiac failure. Pulmonary artery sarcomas tend to be confined to the pulmonary artery, often causing severe right heart failure and metastasize later than right heart sarcomas. They are usually treated with complete resection. Adjuvant therapy is recommended for all patients given that excellent local control is often achieved with surgery, yet long-term survival is often poor due to metastatic recurrence. Cardiac autotransplantation is an excellent technique for resection of posterior or left heart cardiac sarcomas. Surgical outcomes with cardiac autotransplantation are excellent in patients who do not require concurrent pneumonectomy. Pulmonary artery sarcomas allow for radiation therapy in addition to chemotherapy for neoadjuvant control, as the myocardium can be avoided. However, overall, long-term survival after cardiac sarcoma requires improved systemic control. This progress awaits the development of novel chemotherapeutics.