Multifocal Osseous Epithelioid Hemangioendothelioma Involving Extremities and Spine with Visceral Metastasis

Epithelioid hemangioendothelioma (EH) is a rare locally aggressive intermediate grade malignant vasoformative tumor. Bone is a rarer primary site accounting for less than 1% of all malignant bone tumors. Multicentricity and/or multifocality are a relatively frequent occurrence, most commonly clustered in the bones of lower extremities. The axial skeleton is less commonly affected, and concomitant visceral involvement is rarer. We report a unique case of a 20-year-old male who presented with multiple lytic lesions in the skull vault, vertebral bones, bilateral proximal femur, and both radii and humerii with metastasis to the lungs and liver. A final diagnosis of EH was established on biopsy from the right iliac bone. He initially received multiagent chemotherapy and is currently on antiangiogenic therapy. On follow-up positron emission tomography–computerized tomography done 6 months later, marked reduction in fluoro-D-glucose avidity of the hepatic and most skeletal lesions was noted.