Epithelioid Haemengioendothelioma: A Report of Two Cases

Abstract

Epithelioid hemangioendothelioma (EHE), is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar-cell carcinoma. The aetiology is still a dilemma to this day. Studies about suggestive hypothesis are ongoing. Most of the times, it affects lung, liver, and bones, but can arise from any part of the body. It has a very low prevalence of one in one million. Because of its heterogeneous presentation and rarity - represents <1% of all the vascular tumours, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50%–76% of the patients are asymptomatic in the early stage of the disease. A small number of them complain of respiratory symptoms. Bone metastases might cause pathological fractures or cord compression if they arise in vertebrae. Imaging is necessary to determine extend of spread, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli‑1 and CD31 are endothelial‑specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard treatment for EHE.