Diego Chambergo-Michilot
1,2 
, Gabriel De la Cruz-Ku
1,3,4* , Rosalie M. Sterner
3, Ana Brañez-Condorena
5,6 , Pedro Guerra-Canchari
5,7, John Stulak
31 Universidad Científica del Sur, Lima, Perú
2 Department of Cardiology Research, Torres de Salud National Research Center, Lima, Perú
3 Department of Surgery, Mayo Clinic, Rochester, MN, USA
4 Department of Surgery of the University of Massachusetts Medical School, Worcester, MA, USA
5 Universidad Nacional Mayor de San Marcos, Facultad de Medicina, Lima, Perú
6 Asociación de Investigación Estudiantil en Ciencias de la Salud, Lima, Perú
7 Sociedad Científica de San Fernando, Lima, Perú
Abstract
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.