P2.04-046 A Rare Case of Pleuro-Pulmonary Epitheliod Hemangioendothelioma: Topic: Esophageal Cancer and Other Malignancies

Abstract

Epithelioid hemangioendothelioma (EHE) of lung and pleura are rare vascular neoplasm with an epithelioid and histiocytoid appearance that originated from vascular endothelial cells. Pulmonary EHE was first described as “intravascular bronchioloalveolar tumor” (IVBAT) by Dail et al. in 1975, since it was believed to be an aggressive form of bronchoalveolar cell carcinoma with propensity to invade adjacent blood vessels. It is described by the World Health Organization 2002 classification as lesion locally aggressive with metastatic potential, a neoplasm of low to intermediate-grade malignancy. It occurs with a predilection for younger (mean age 39) female (60% of cases). Most patient are asymptomatic (50%) although dyspnea, cough, chest pain, hemoptysis can occur. By immunohistochemistry, EHE shows the typical markers of vascular differentiation. Primary epithelioid hemangioendotheliomas of pleura are extremely rare, usually affecting males, and associated with a variety of clinical manifestations and poor prognosis. We present a rare case of EHE with an extensive pleuro-pulmonary involvement. A 50-year old non-smoking woman presented to our institution complaining of persistent cough and progressive dyspnea first treated with antibiotics without improvement of the symptoms. Chest computed tomography (CT) showed multiple disseminated nodules of both lungs mostly involving the upper lobes with associated right-sided pleural effusion and thickening. There is a elevated serum level of Cancer Antigen (CA) 125. In a right triportal video-assisted thoracoscopy surgery (VATS) we performed wedge resections of the right upper, middle and lower lobes with diffuse nodular process and multiple biopsies of thickened pleura. Pathological examination of the pleuro-pulmonary samples showed multiple, diffuse nodular infiltrates of epithelioid cells with frequent cytoplasmic vacuoles, rare mitoses and foci of tumor necrosis. Immunohistochemistry was positive for Vimentin, CD31, CD34, ETS-related gene (ERG). Therefore, a final diagnosis of Epithelioid hemangioendothelioma was made on the basis of the radiographic, cytomorphologic, and immunohistochemical findings. Pulmonary Epithelioid hemangioendothelioma is a vascular tumor with low to intermediate-grade malignancy. Pleural epithelioid hemangioendothelioma is less common and the clinical behavior is more aggressive and has a poorer prognosis. Our case is an epithelioid hemangioendothelioma with a clinical and pathologic pleuro-pulmonary involvement and a very aggressive clinical course.