Abstract Body (Do not enter title and authors here): Cardiac Rhabdomyosarcoma (CR) is a rare malignant neoplasm of the heart occurring in all age groups and genders. It can arise anywhere in the heart with varied clinical presentation. Available data on CR are mainly from case reports and series that are limited by small sizes. The purpose of this study was to characterize the epidemiology, presentation, management, hospital course, and outcomes of CR published in the English literature from 1980-2023.

Methods:
We reviewed and included all published case reports with a diagnosis of CR in adult patients (age >18 years) on PubMed and Google Scholar using the keywords “primary cardiac tumor” and “cardiac rhabdomyosarcoma”. Data was extracted onto an Excel spreadsheet for analysis and the outcomes of interest were demographics, clinical presentation, diagnostic modalities, management, complications, and outcomes. We used descriptive statistics to analyze the data.

Results:
Among a total of 97 patients from 19 countries included in this study, 54% were males and the median age was 49 years (range 18-80). The main symptoms were dyspnea (37.1%), palpitations (23.7%), chest pains (16.5%) and syncope (6.5%). Hypotension or heart failure and arrhythmias were present in 10.3% and 9.3%, respectively and 5.2% had pericardial effusion. In addition to those with metastatic tumors, 22.7% had chronic co-morbidities. The majority of the cases were primary tumors (85.6%), and the remainder were (14.4%). All patients had at least one imaging modality including echocardiography (89%) and CT/MRI (84%). The commonest sites of cardiac involvement were the left atrium (35%), right atrium (33%), right ventricle (20%), and left ventricle (12%). Approximately 68% involved a single cardiac site while 32% involved multiple sites. Among the patients, 96% underwent surgery while 33% and 25% had chemotherapy and 25% radiotherapy, respectively. The median length of hospital stay was 11 days (range, 2-68 days), and mortality was recorded in 79.3% (77/97) of the patients. The median survival time was reported in 42 patients, and it was 6 months (range, 0-36 months).


Conclusion:
Cardiac rhabdomyosarcoma is a rare and aggressive malignant neoplasm. Most cases are primary tumors with poor outcomes such as a very short median survival time and a very high mortality rate despite the combination of surgery, chemotherapy, and radiotherapy. This calls for further research into the early diagnosis and optimal management strategies to improve outcomes.