Tabriz University of Medical Sciences Journal of Cardiovascular and Thoracic Research 2008-5117 15 1 2023 03 16 Clinical characteristics, management, and outcomes of patients with primary cardiac angiosarcoma: A systematic review 1 8 10.34172/jcvtr.2023.30531 EN Diego Chambergo-Michilot https://orcid.org/0000-0002-3904-7514 Gabriel De la Cruz-Ku https://orcid.org/0000-0003-4203-3890 Rosalie M. Sterner Ana Brañez-Condorena https://orcid.org/0000-0001-5518-3025 Pedro Guerra-Canchari John Stulak REVIEW 10.34172/jcvtr.2023.30531 2022 02 23 2023 02 10 Primary cardiac angiosarcomas (PCA) are highly aggressive malignant heart tumors. Previous reports have shown a poor prognosis regardless of management, and no consensus or guidelines exist. It is necessary to clarify this information since patients with PCA have a short survival. Therefore, we aimed to systematically review clinical manifestations, management, and outcomes. We systematically searched in PubMed, Scopus, Web of Science, and EMBASE. We intended to include cross-sectional studies, case-control studies, cohort studies, and case series that reported clinical characteristics, management, and outcomes of patients with PCA. As a methodological approach, we used the Joanna Briggs Institute Critical Appraisal Checklist for Case Series and the Newcastle-Ottawa Scale for cohorts. We included six studies (five case series, one cohort). The mean/median age ranged from 39 to 48.9 years. Male sex was predominant. The most frequent manifestations were dyspnea (range: 50%-80%), pericardial effusion (29% & 56%), and chest pain (10%-39%). The mean tumor size ranged from 5.8 to 7.2 cm, with the majority of these localized in the right atrium (70-100%). The most common locations of metastasis were the lung (20%-55.6%), liver (10%-22.2%), and bone (10%-20%). Resection (22.9%-94%), and chemotherapy as neoadjuvant or adjuvant (30%-100%) were the most commonly used methods of treatment. Mortality ranged from 64.7% to 100%. PCA often presents late in its course and usually results in poor prognosis. We strongly recommend performing multi-institutional prospective cohorts to better study disease course and treatments to develop consensus, algorithms, and guidelines for this type of sarcoma.