Epithelioid hemangioendothelioma of bone--a clinicopathologic and immunohistochemical study of 7 cases
Epithelioid hemangioendothelioma is a rare vascular tumor of bone. Between 1987-1999, seven cases of epithelioid hemangioendothelioma were recorded. The histopathological evaluation was done with hematoxylin and eosin stain and immunohistochemical stains along with the analysis of clinical data and X-ray findings. There were 4 males, 3 females between 15 to 48 years of age with complaints of pain and swelling. Long bones were involved in five of the seven patients and one was multifocal. Radiologically all seven patients showed osteolytic lesions. The histologic hall mark was the presence of eosinophilic, epithelioid cells with intracytoplasmic vacuoles. The tumor may be histologically confused with metastatic carcinoma, chondromyxoid fibroma and osteogenic sarcoma. Immunohistochemical reactivity was as follows: Vimentin (6/6 cases), Factor VIII related antigen (4/6 cases), Ulex Europeaus (5/6 cases), CD31 (3/5 cases), CD34 (3/5 cases), epithelial membrane antigen (1/6 cases), cytokeratin (none). Treatment comprised curettage (4 cases), wide excision (2 cases), below knee amputation (1 case) and post operative radiotherapy (2 cases). At follow-up, (available in 4 patients) two were without disease at 18 months and 120 months. Of the remaining two, one developed local recurrence after 36 months; while the other died of lung metastasis (18 months). A lytic bone shadow, the presence of plump eosinophilic often vacuolated cells on a myxoid background and immunohistochemistry together help in the correct recognition of this tumor.