Objective: To describe the clinical features of pulmonary epithelioid hemangioendothelioma.
Methods: With a case report and review of the related literatures, the etiology, clinical manifestations, diagnosis, differential diagnosis, management and prognosis of pulmonary epithelioid hemangioendothelioma were described.
Results: The etiology of this rare disease remains unknown. Symptoms are scanty and usually mild; chest radiograph or computed tomography usually reveals multiple bilateral pulmonary nodules. Primitive lumen formed by a single cell is the pathologic feature. Immunohistochemical stains show that the malignant cells are of the endothelial type. There is no effective treatment for this disease and its prognosis is unpredictable.
Conclusion: Pulmonary epithelioid hemangioendothelioma is rare and often misdiagnosed as other pulmonary diseases.