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Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor exceptionally involving the pleura with less than 30 cases reported in literature. We herein describe another case of pleural EHE in a 79-year-old man with medical history of chronic obstructive pulmonary disease and high blood pressure. He presented rightsided pleural effusion. Computerized tomography revealed multifocal pleural thickening and effusion. Pleural biopsy was performed. Microscopically, the tumor showed a biphasic pattern with cords and nests of epithelioid cells showing mild atypia and rare mitosis with intracytoplasmic lumina containing red blood cells. The second pattern is composed of spindle-shaped cells with occasional necrosis. The tumor cells were positive for CD34 and focally with CK7. The diagnosis of EHE was made.
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