Bone Pathology Database

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2nd image
Lateral X ray
Third image
Detail of lesion in the femoral diaphysis
The lesions are lytic and "punched out" type with a fairly well defined margin.The patient had no lymphademopathy or other palpable masses. X rays showed all long bones were affected as well as flat bones such as the pelvis. The spinous processes of the spine were also affected. Serum protein electrophoresis was normal. A technicium scan showed diffuse uptake corresponding to the sites of the lesions. Histology of the tibial site showed an endotheliohemangioma.

Epithelioid hemangioendothelioma is a rare, well-differentiated endothelial tumor with a wide spectrum of behavior. It consists of a varied group of proliferative and neoplastic vascular lesions. The tumor has a biological behavior which falls somewhere between the benign hemangioma and malignant angiosarcoma. Approximately 10% of cases are associated with other developmental anomalies or syndromes, including early onset varicose veins, lymphedema, Klippel-Trenaunay-Weber syndrome, and Maffucci's syndrome. Epithelioid hemangioendothelioma occurs in the calvarium, spine, femur, tibia and feet of adults during the second or third decade. This case is atypical because of the patient is much older. The lesion is often multiple in the same bone or diffuse as in this example.

Record 105