Pulmonary Epithelioid Hemangioendothelioma: Advances in Treatment Options despite a Rare Vascular Tumor

We have already discussed about dail and liebow describing pulmonary epithelioid hemangioendothelioma (PEH) as an aggressive bronchoalveolar cell carcinoma with a remarkable propensity to invade adjacent blood vessels and small airways, the following Weiss and Enzinger description of epithelioid hemangioendothelioma (EHE) as a vascular bone and soft tissues tumor showing intermediate malignancy between hemangioma and angiosarcoma, and the final confirmation from Weldon-Linne of a factor-VIII-related antigen onto malignant cells [1]. The recent World Health Organization (WHO 2002) classification has described EHE as lesions that fall into the category of locally aggressive tumors with metastatic potential [2,3]. The etiology of EHE is still a dilemma, as several clonal abnormalities in tumor cells, and different angiogenic stimulators may act as promoters of endothelial cell proliferation [4-8]. A new etiopathogenetic hypothesis suggests a causal relationship between chronic Bartonella infection and the development of this rare vascular tumor: if this was confirmed, it would be plausible that eradicating the bacterial infection or interrupting Bartonella-induced angiogenic and proliferative cell signals could slow the tumor progression and improve patient outcomes [9].