Clinical characteristics, management, and outcomes of patients with primary cardiac angiosarcoma: A systematic review

Diego Chambergo-Michilot, Gabriel De la Cruz-Ku, Rosalie M. Sterner, Ana Brañez-Condorena, Pedro Guerra-Canchari, John Stulak

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


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.

Original languageEnglish
Pages (from-to)1-8
Number of pages8
JournalJournal of Cardiovascular and Thoracic Research
Issue number1
StatePublished - Mar 2023


  • Angiosarcoma
  • Heart
  • Hemangiosarcoma
  • Malignant Haemangioendothelioma
  • Systematic review


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