A Rare Primary Lung Tumor: Pulmonary Epithelioid Hemangioendothelioma and a Literature Review

Abstract

Epithelioid hemangioendothelioma is an extremely rare vascular tumor, with a prevalence of less thanone in one million each year. It is most commonly described in the veins of the extremities, but it mayalso arise from the vascular endothelial or pre-endothelial cells of the lung. Most of the patients areyoung women. The clinical behavior of pulmonary endotheloid hemangioma (PEH) ranges from welldifferentiatedhemangioma to high-grade angiosarcoma. Chest CT is characterized by the presence of anodule that is either single or multiple in one lung or both. For the diagnosis, a good histopathologicalevaluation of the biopsy is required. Depending on the rarity of the disease, there is no standardizationin treatment. Chemotherapy can be administered for widespread disease, with unclear benefits. Drugsthat block the vascular endothelial growth factor are expected to be effective with a small number ofpromising case reports. What we know about the disease and how to manage is all those derived fromapproximately 250 case reports. Thus, we aimed to present one more patient with bilateral pulmonaryepithelioid hemangioma with two years follow up results after some chemotherapy.