Background: Primary tumors of the heart are rare even in major cardiac surgery centers. Because of the low case numbers, there is an insufficient evidence base to determine the optimal treatment, particularly for malignant tumors.
Method: The authors review the pertinent literature retrieved by a selective PubMed search on the terms "cardiac tumor," "heart tumor," "cardiac myxoma," and "cardiac sarcoma." They also present operative techniques and their own long-term results in 181 patients with cardiac tumors.
Results: Patients with cardiac tumors generally have nonspecific symptoms depending on the site of the tumor and the extent of infiltration into the neighboring tissue. The diagnosis is based on the clinical history, echocardiography (in most cases), and, sometimes, computerized tomography and magnetic resonance imaging. Autopsy studies reveal a 0.02% prevalence of cardiac tumors, of which 75% are benign and 25% malignant. Myxoma is the most common benign tumor (50-70%); angiosarcoma is the most common malignant one (30%), followed by rhabdomyosarcoma (20%). About 10% of all tumor patients develop cardiac metastases, but these are only rarely clinically manifest. From 1989 to 2012, 181 patients underwent surgery for cardiac tumors in the authors' institution. The 5-year survival rates were 83% for benign tumors (139 patients), 30% for malignant tumors (26 patients), and 26% for cardiac metastases (16 patients).
Conclusion: Patients with cardiac tumors should undergo surgery in a timely fashion in a specialized center. This holds for both malignant and benign tumors, particularly for atrial myxoma, which can cause serious secondary complications by embolization.