Pleural Epithelioid Hemangioendothelioma: A Case Report and Literature Review

Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin that can arise in multiple and varied tissue sites. Pleural epithelioid hemangioendothelioma (PEH), a subtype of EHE, is particularly less reported. Herein, we describe a case of PEH presented with left-sided back pain in a 68-year-old female, and her chest CT scan revealed thickening of the left pleura and left pleural effusion, the histological diagnosis was confirmed by both conventional examination and immunohistochemistry. A literature search utilizing PubMed, Embase, Ovid and Cochrane, Wanfang and Chinese National Knowledge infrastructure (CNKI) for PEH was conducted to investigate the characteristics of the disease, 26 related articles were retrieved and 40 cases of PEH were reported. According to available literature, the average age at presentation is 51.8 years and the disease occurred more often in men than women. The etiology of the disease remained unknown. Chest pain, cough, and dyspnea were the common symptoms. Computed tomography usually revealed pleural effusion and pleural thickening. Histological examinations revealed mainly epithelioid cells. Immunohistochemical stains were positive for vascular endothelial markers. PEH tends to have more aggressive behavior than tumors in other locations, thus effective treatment has not yet been established until now. Further studies are needed to analyze the prognostic factors, clinical features and treatment of PEH.