Abstract
Introduction: Primary cardiac angiosarcoma is an extremely rare and aggressive malignant neoplasm with an estimated incidence of 0.017% among cardiac tumors. It predominantly occurs in the right atrium and has a clinical course characterized by rapid infiltration and early metastasis. Its initial manifestations are often nonspecific, making early diagnosis difficult, which results in a poor prognosis and a median survival of less than one year. Objective: To systematically analyze the clinical, diagnostic, therapeutic, and evolutionary characteristics of cardiac angiosarcoma based on reports and case series published between 2020 and 2025. Methodology: A systematic review was conducted following PRISMA guidelines. The search was performed in PubMed using MeSH descriptors and Boolean operators. Only reports and case series with confirmed histopathological diagnosis were included, resulting in 18 studies selected for narrative analysis. Results: The analyzed cases showed a predominance of primary tumors in the right atrium, mainly affecting young or adult males. The most used diagnostic techniques were echocardiography, computed tomography, and magnetic resonance imaging. Complete surgical resection was performed in 11 patients; however, the recurrence rate was high, and first-year mortality remained significant. Discussion: Addressing cardiac angiosarcoma continues to be a clinical challenge due to its late detection and therapeutic limitations. The integration of diagnostic tools and timely surgical decisions improves management but does not always change the outcome. Conclusion: Given the aggressiveness of this tumor, having an early diagnosis, a comprehensive clinical approach, and more evidence accumulation is essential to improve prognosis and decision-making in medical practice.
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