A patient with a pleural epitheloid hemangio-endothelioma (EHE) who failed to respond to six cycles of initial chemotherapy with iphosphamide and epirubicine was treated with pazopanib in second-line. A significant subjective and objective metabolic response on (18)F-fluoro-deoxyglucose positron-emission tomography-computed tomography was noted. Based on this observation, the role of vasculoendothelial growth factor receptor inhibitors such as pazopanib (or other tyrosine kinase inhibitors), in the treatment of pleural EHE should be established through prospective collaborative studies as upfront medication and in combination with chemotherapy.
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