Epithelioid hemangioendothelioma is a rare vascular tumor. Most commonly it affects lung, liver and bones, although it may also involve the head and neck region, breast, lymph nodes, brain, skin and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not treated correctly since there are no standard treatment protocols, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. Imaging is necessary to know the involvement of surrounding tissues. 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. We focused on Radiation treatment as a good therapeutic option despite the poor prognosis, radiotherapy offers local pain control with good tolerance and better quality of life at least at a one-year followup in most of cases. Further studies are needed to establish the standard radiation dose to be used for better locoregional control of such a complex and extremely rare disease.
We report a case of a woman who found to have multiple bone and hepatic masses on imaging, initially misdiagnosed as a metastatic breast carcinoma presented with breast and axillary mass with subcutaneous nodules on forearm. The diagnosis of Epithelioid hemangioendothelioma was made on histopathological study and confirmed by immunohistochemistry, which showed diffuse response for CD31,CD34 markers and no response to tissue CEA, HMB-45 or S-100 protein.