Characteristics, survival and incidence rates and trends of primary cardiac malignancies in the United States

Background: The available literature on the incidence, management and prognosis of primary malignant cardiac tumors [PMCTs] is limited to single-center studies, prone to small sample size and referral bias. We used data from the Surveillance, Epidemiology, and End Results [SEER]-18 registry (between 2000 and 2014) to investigate the distribution, incidence trends and the survival rates of PMCTs. Methods: We used SEER*Stat (version 8.3.4) and the National Cancer Institute's Joinpoint Regression software (version 4.5.0.1) to calculate the incidence rates and annual percentage changes [APC] of PMCTs, respectively. We later used SPSS software (version 23) to perform Kaplan-Meier survival tests and covariate-adjusted Cox models. Results: We identified 497 patients with PMCTs, including angiosarcomas (27.3%) and Non-Hodgkin's lymphomas [NHL] (26.9%). Unlike the incidence rate of NHL (0.108 per 106 person-years) that increased significantly (APC=3.56%, 95% CI, [ 1.445 to 5.725], P=.003) over the study period, we detected no significant change (APC=1.73%, 95% CI [-3.354 to 7.081], P=.483) in the incidence of cardiac angiosarcomas (0.107 per 106 person-years). Moreover, our analysis showed that the overall survival of NHL is significantly better than angiosarcomas (P<.001). In addition, surgical treatment was associated with a significant improvement (P=.027) in the overall survival of PMCTs. Conclusion: Our analysis showed a significant increase in the incidence of cardiac-NHL over the past 14 years with a significantly better survival than angiosarcomas. To further characterize these rare tumors, future studies should report data on the medical history and diagnostic and treatment modalities in these patients. (c) 2017 Elsevier Inc. All rights reserved.