Original Article Characteristics, survival and incidence rates and trends of primary cardiac malignancies in the United States ☆ Anas M. Saad a,1 , Abdelrahman Ibrahim Abushouk a, ⁎ ,1 , Muneer J. Al-Husseini a , Sami Salahia a , Anas Alrefai a , Ahmed M. Afifi a , Mohamed M. Abdel-Daim b,c a Faculty of Medicine, Ain Shams University, Cairo, Egypt b Pharmacology Department, Suez Canal University, Ismailia, Egypt c Department of Ophthalmology and Micro-Technology, Yokohama City University, Yokohama, Japan abstract article info Article history: Received 23 November 2017 Received in revised form 17 December 2017 Accepted 20 December 2017 Available online xxxx 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 lympho- mas [NHL] (26.9%). Unlike the incidence rate of NHL (0.108 per 10 6 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 10 6 person-years). Moreover, our analysis showed that the overall survival of NHL is significantly better than angiosarcomas (Pb.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. © 2018 Elsevier Inc. All rights reserved. Keywords: Epidemiology Heart Surveillance Tumors 1. Introduction Cardiac tumors (whether benign or malignant) are rare events and the literature on their incidence, management and prognosis is quite limited [1]. In the majority of published autopsy series, metastatic car- diac tumors are more frequent than primary tumors by a 30-to-1 ratio [2]. According to the medical literature, the incidence of primary cardiac tumors in the general population varies between 0.001% and 0.03%, representing only 0.3% of all open-heart surgeries [3]. The available data on such tumors come primarily from single-center studies, which can be limited by their small sample size and referral bias. Fortunately, the majority of primary cardiac tumors are benign with myxomas representing 80% of cases [4]. Primary malignant cardiac tumors [PMCTs] are mainly sarcomas and to a lesser extent primary cardiac lym- phomas [5–7]. These tumors only manifest when they obstruct the intra- cardiac flow or interfere with the valvular components or the conducting system [8]. Therefore, the diagnosis of cardiac malignancy is a complicated process that requires clinical suspicion [9]. While the transthoracic echo- cardiography (TEE) is the first choice for the diagnosis of cardiac tumors, computed tomography (CT) and magnetic resonance imaging (MRI) are useful for characterization and differential diagnosis [10,11]. Considering the lack of data in the literature on PMCTs, our main ob- jective in this study was to provide an in-depth analysis of the distribu- tion, trends in the incidence, and survival (overall and cancer-specific) of these tumors. For that, we used a large patient cohort from the SEER database, during the period from 2000 to 2014. Cardiovascular Pathology 33 (2018) 27–31 Abbreviations: APC, Annual percentage changes; NHL, Non-Hodgkin's lymphoma; PMCTs, Primary cardiac malignant tumors; SEER, Surveillance, Epidemiology and End- Results. ☆ Conflict of interestThe authors declare no potential conflicts of interest, including em- ployment, consultancies, stock ownership, honoraria, paid expert testimony, patent appli- cations/registrations, and grants or other funding. ⁎ Corresponding author at: Faculty of Medicine, Ain Shams University, Cairo, 11591, Egypt. Tel./fax: +20 1014295780. E-mail address: Abdelrahman.abushouk@med.asu.edu.eg (A.I. Abushouk). 1 Both authors contributed equally. https://doi.org/10.1016/j.carpath.2017.12.001 1054-8807/© 2018 Elsevier Inc. All rights reserved. Contents lists available at ScienceDirect Cardiovascular Pathology