A case report of right atrial epithelioid hemangioendothelioma with multiple pulmonary metastases

Cardiac epithelioid hemangioendothelioma (EHE) is a very rare tumour of endothelial origin with the lung and liver as the most easily metastatic organs. We describe herein a patient with hemoptysis, severe anaemia, and diffuse pulmonary nodules with halo signs that represented metastasis of cardiac EHE; these radiologic manifestations are relatively uncommon. During the initial workup for the patient's pulmonary nodules, echocardiography missed the cardiac mass. However, positron emission tomography-computed tomography revealed increased fluorodeoxyglucose intake in the right atrial wall, and cardiac magnetic resonance imaging (MRI) revealed an irregular nodule with normal T1-weighted signal intensity and hyperintense T2-weighted signal intensity. Enhanced abdominal computed tomography (CT) revealed micronodular liver metastases. Video-assisted thoracic surgery was performed to make a definitive diagnosis. Immunohistochemistry staining proved the diagnosis of EHE with positive results for cluster of differentiation (CD) 34, CD31, erythroblast transformation-specific-related gene and Ki-67. The patient started chemotherapy with docetaxel (75 mg/m(2)) and gemcitabine (900 mg/m(2)), but this failed to control his disease and he died from an opportunistic infection related to his immunocompromised status 5 months later. For the work out process of bilateral diffuse pulmonary nodules suspicious for cardiac origin, especially with atrial deviation, echocardiography alone is not sufficient to exclude atrial origin. Cardiac CT or MRI might be a better choice.