Abstract: Objective To understand further and improve the diagnostic accuracy of pulmonary epithelioid hemangioendothelioma. MethodsWe analyzed the etiology, clinical manifestations, diagnosis,differential diagnosis, management and prognosis of pulmonary epithelioid hemangioendothelioma after diagnosing one case and reviewing some literatures. ResultsThe etiology remained unknown,and symptoms were scanty and usually mild. Chest X-ray or computed tomography usually revealed multiple nodules in bilateral pulmonary. Pleural effusions emerged due to pleura was invaded. Primitive lumen formed by a single cell was the pathological feature. Immunohistochemical stains showed that the cells were original from the endothelial type. There is no effective treatment for this disease and its prognosis is in between benign lession and malignant neoplasm. ConclusionPulmonary epithelioid hemangioendothelioma with bilateral pleural effusion is rare and often misdiagnosed as other pulmonary diseases.

Key words: hemangioendothelioma,epithelioid, lung neoplasms, diagnosis