Pulmonary Artery Intimal Sarcoma with Acute PE and Right Atrial Involvement
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Imaging findings
PE protocol CT shows extensive bilateral pulmonary emboli, completely blocking the right main pulmonary artery, with signs of acute right ventricular strain (dilated pulmonary artery, increased right atrial/ventricular diameters, septal bowing). An unusual nodular-like soft tissue mass is seen hanging along the free wall of the right atrium. Lung nodules are also present. CT angiogram shows a mass extending into and occluding the right pulmonary artery, confirmed to be sarcoma with superimposed thrombus, after surgical endarterectomy.
Key takeaways
Pulmonary artery intimal sarcoma can mimic chronic thromboembolic pulmonary hypertension or acute pulmonary embolism, but its nodular, often enhancing appearance within the vessel and potential extension into the heart (right atrium) or lung parenchyma are distinguishing features. Histopathology often reveals an undifferentiated sarcoma.
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