clinical characteristics, management, and outcomes of patients with primary cardiac angiosarcoma: a systematic review

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.