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Primary Pericardial Angiosarcoma

Imaging findings

Contrast-enhanced chest CT shows a large, lobulated, highly vascular anterior mediastinal mass centered in the pericardium, resulting in severe right atrial compression and cardiac tamponade. Multiple highly vascular metastatic nodules containing prominent internal vessels are visualized throughout both lungs. Gross pathological inspection at autopsy confirmed a primary pericardial angiosarcoma arising purely from the pericardium with no epicardial fat component, alongside hemorrhagic, black-red metastatic nodules covering the lung parenchyma.

Key takeaways

Primary cardiac angiosarcomas are rare, highly aggressive malignant vascular tumors that typically arise from the right atrium or the epicardial fat. This case demonstrates an unusual variant arising strictly from the pericardium without epicardial fat involvement. Due to the extreme vascularity of these neoplasms, biopsy or surgical manipulation carries a catastrophic risk of uncontrollable hemorrhage, hemopericardium, and fatal tamponade.

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