Cardiac tumors.

Primary cardiac tumors are rare but have the potential to cause significant morbidity if not treated in an appropriate and timely manner. To date, however, there have been no studies examining survival characteristics of patients who undergo surgical resection. From 1957 to 2006, 323 consecutive patients underwent surgical resection of primary cardiac tumors; 163 (50%) with myxomas, 83 (26%) with papillary fibroelastomas, 18 (6%) with fibromas, 12 (4%) with lipomas, 28 (9%) with other benign primary cardiac tumors, and 19 (6%) with primary malignant tumors. Operative (30 day) mortality was 2% (n=6). Univariate analysis indicated that patients who underwent resection of fibromas and myxomas had superior survival characteristics in comparison to the remainder of tumor variants; these results were consistent after adjusting for age at surgery, year of surgery, and cardiovascular risk factors. Based on actuarial characteristics of the 2002 U.S. population, patients who underwent myxoma resection had survival characteristics that were not significantly different from that of an age and gender matched population (SMR 1.11, P=0.57) whereas those who underwent resection of fibromas (SMR 11.17, P=0.002), papillary fibroelastomas (SMR 3.17, P=0.0003), lipomas (SMR 5.0, P=0.0003), other benign tumors (SMR 4.63, P=0.003), and malignant tumors (SMR 101, P<0.0001) had significantly poorer survival characteristics. Furthermore, malignant tumors in younger patients were highly fatal (HR 0.899, P<0.0001). Although the most significant predictor of mortality was tumor histology, survival was also influenced the by the duration of CPB and NYHA III/IV; the impact of these risk factors varied with time. The cumulative incidence of myxoma recurrence was 13% and occurred in a younger population (42 versus 57 years, P=0.003) with the risk of recurrence decreased after 4 years. Surgical resection of primary cardiac tumors is associated with excellent long-term survival; patients with cardiac myxomas have survival characteristics that are not significantly different from that of a general population. Predictors of mortality are primarily related to tumor histology but also include clinical characteristics such as symptomatology and duration of CPB.

Between March 1980 and September 1997, 91 patients underwent evaluation and treatment for primary cardiac neoplasms. Tumors were grouped into three categories: atrial myxomas, benign nonmyxomas, and malignant tumors. Survivors were contacted; no one was lost to follow-up. The mean follow-up for this series is 7 +/- 5 years. Eighty-three patients were diagnosed with atrial myxomas (Male/Female: 29/54), average age 55 +/- 13 years. The hospital mortality was 3.6% (3/83), the late mortality was 6.5% (5/80). No recurrent myxomas have been identified clinically or by echocardiography in any patient. Three patients were diagnosed with benign nonmyxoma tumors. (Male/Female: 2/1), average age 64 +/- 8 years. There were no perioperative deaths and 1 patient died 4 years postoperatively from fibroma, with no linked causes. No recurrent tumors have been identified. Five patients were diagnosed with malignant tumors. (M/F: 1/4), average age 53 +/- 16 years. The hospital mortality was 20% (1/5); in 3 patients a redo-operation was necessary after 8, 11, and 12 months because of tumor recurrence. All patients died within 3 years of the first operation (mean 13 +/- 14 months). Surgical resection, when possible, is the treatment of choice for all primary cardiac tumors. Patients with benign tumors are probably cured by resection and in our experience there was no known tumor recurrence. Effective palliation is possible with resection of malignant tumors, but more effective adjuvant therapy will be necessary to improve long-term prognosis.

Primary cardiac sarcomas (PCS) are rare tumours of dismal prognosis.