Epithelioid hemangioendothelioma of bone. A clinicopathologic, ultrastructural, and immunohistochemical study

Abstract

In a review of 29 cases of solitary and multicentric hemangioendothelial sarcomas of bone, 14 were found to have histologic features of epithelioid hemangioendotheliomas. These were characterized by the presence of epithelioid or "histiocytoid" endothelial cells that were either round or spindle-shaped. Intracytoplasmic vacuolization was noted, and some showed nesting of cells, mimicking metastatic adenocarcinoma. The presence of a myxoid matrix suggested the diagnosis of chondrosarcoma in some cases. Some of the tumors strongly resembled the lesions formerly designated as intravascular bronchioloalveolar tumor, angioglomoid tumor of bone, or malignant myxoid angioblastoma of bone. Factor VIII-related antigen was detected in five of the six cases examined, and ultrastructural study in five cases confirmed the endothelial nature of the tumor cells; Weibel-Palade bodies were present in three cases. There were 11 men and three women, and the tumors were seen predominantly in patients who were under 30 years of age (10 cases). Four cases involved solitary tumors, and nine were multicentric in bone. The multicentric tumors had a predilection for the bones of one lower extremity (five cases). The total series had a protracted clinical course, and the multicentric tumors appeared to follow a less aggressive course.