A Rare Primary Lung Tumor: Pulmonary Epithelioid Hemangioendothelioma and a Literature Review
Hülya DİROL1,Ömer ÖZBUDAK1,İrem Hicran ÖZBUDAK2
1Department of Chest Diseases, Akdeniz University, Antalya-Turkey
2Department of Pathology, Akdeniz University, Antalya-Turkey
DOI : 10.5505/tjo.2019.2113 Epithelioid hemangioendothelioma is an extremely rare vascular tumor, with a prevalence of less than one in one million each year. It is most commonly described in the veins of the extremities, but it may also arise from the vascular endothelial or pre-endothelial cells of the lung. Most of the patients are young women. The clinical behavior of pulmonary endotheloid hemangioma (PEH) ranges from welldifferentiated hemangioma to high-grade angiosarcoma. Chest CT is characterized by the presence of a nodule that is either single or multiple in one lung or both. For the diagnosis, a good histopathological evaluation of the biopsy is required. Depending on the rarity of the disease, there is no standardization in treatment. Chemotherapy can be administered for widespread disease, with unclear benefits. Drugs that block the vascular endothelial growth factor are expected to be effective with a small number of promising case reports. What we know about the disease and how to manage is all those derived from approximately 250 case reports. Thus, we aimed to present one more patient with bilateral pulmonary epithelioid hemangioma with two years follow up results after some chemotherapy. Keywords : Pulmonary epitheloid hemangioendothelioma; vascular endothelial growth factor-A (VEGF-A), weibelpalade body