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

07:00 EST 3rd December 2019 | BioPortfolio

Summary of "A case report of right atrial epithelioid hemangioendothelioma with multiple pulmonary and liver metastases."

Cardiac epithelioid hemangioendothelioma is a very rare tumor of endothelial origin with the lung and liver as the most easily metastatic organs. We describe herein a patient with hemoptysis, severe anemia, 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 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 ) and gemcitabine (900 mg/m ), 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.

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This article was published in the following journal.

Name: The clinical respiratory journal
ISSN: 1752-699X
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Medical and Biotech [MESH] Definitions

A tumor of medium-to-large veins, composed of plump-to-spindled endothelial cells that bulge into vascular spaces in a tombstone-like fashion. These tumors are thought to have "borderline" aggression, where one-third develop local recurrences, but only rarely metastasize. It is unclear whether the epithelioid hemangioendothelioma is truly neoplastic or an exuberant tissue reaction, nor is it clear if this is equivalent to Kimura's disease (see ANGIOLYMPHOID HYPERPLASIA WITH EOSINOPHILIA). (Segen, Dictionary of Modern Medicine, 1992)

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A type of cardiac arrhythmia with premature atrial contractions or beats caused by signals originating from ectopic atrial sites. The ectopic signals may or may not conduct to the HEART VENTRICLES. Atrial premature complexes are characterized by premature P waves on ECG which are different in configuration from the P waves generated by the normal pacemaker complex in the SINOATRIAL NODE.

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