Right Atrial Epithelioid Hemangioendothelioma
Imaging findings
Echocardiography and CT show a highly lobulated, frond-like, mobile mass in the right atrium extending through the Thebesian valve into the coronary sinus and IVC. Staging and surgical embolectomy confirmed right atrial epithelioid hemangioendothelioma with associated pulmonary tumor emboli mimicking CTEPH.
Key takeaways
Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm of intermediate malignancy. When arising in the right atrium, it can mimic a benign myxoma or simple thrombus, leading to chronic pulmonary tumor emboli and pulmonary hypertension.
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