Therapy Insight: Malignant primary cardiac tumors

Benign cardiac tumors are resected with a high degree of success with modern cardiac surgical techniques. Malignant cardiac tumors, however, continue to pose a therapeutic challenge to cardiac surgeons and oncologists because of the technical difficulty involved in extensive cardiac resections and the aggressive biological nature of the tumors. The majority of malignant cardiac tumors are sarcomas and can be categorized as right heart sarcoma, left heart sarcoma or pulmonary artery sarcoma. Right heart sarcomas are generally angiosarcomas, which infiltrate widely and metastasize early. A combination of chemotherapy and surgical resection is the preferred therapy. Left heart sarcomas, although large, are often less infiltrative and metastasize later than right heart sarcomas, but a similar approach to treatment is usually employed. Surgical resection is more-frequently necessary for left heart sarcomas because of intracardiac blood flow obstruction and congestive heart failure, although the anatomic position and relation of these tumors to cardiac structures can complicate surgery. We have developed and employed the technique of cardiac autotransplantation, which involves cardiac excision, ex vivo tumor resection with cardiac reconstruction, and cardiac reimplantation, to lessen these technical difficulties. Pulmonary artery sarcomas can be treated by radiotherapy, as well as by the other therapies, because the myocardium can be avoided by the radiation fields. Surgical resection of this sarcoma type often requires pneumonectomy and can require pulmonary root replacement.