A 48-year-old male patient presented with progressive scalp swelling over the past year. Clinical examination revealed a large bony lobulated ulcerated swelling involving the scalp. Skull radiograph revealed ill-defined, permeative lytic destruction of the calvarium accompanied by a large soft-tissue mass (Figure, A). Contrast-enhanced CT confirmed these bony findings, with an associated heterogeneously enhancing soft-tissue mass (Figure, B–D). A provisional diagnosis of calvarial osteosarcoma was considered. Subsequent core needle biopsy revealed cells with epithelioid morphology that were positive for ERG, CD31, and CD34, markers indicative of epithelioid hemangioendothelioma (EHE). The patient was started on pazopanib therapy.
A 48-year-old male patient with scalp swelling. (A) Frontal skull radiograph reveals a permeative lytic calvarial lesion with large associated soft tissue (asterisk). (B) Contrast-enhanced head CT scan (soft-tissue window) additionally shows a large heterogeneously enhancing soft-tissue mass (asterisk). (C, D) Sagittal and axial CT bone window images better demonstrate the permeative lytic bone destruction of the calvarium (arrows) by the mass.
EHE is a rare, locally aggressive vascular tumor with malignant potential, originating from diverse sites in the body, most commonly the liver (21%), bones (14%), and lung (12%) (1,2). Osseous EHE may affect both long bones and the axial skeleton (3). Radiologically, it is an osteolytic lesion devoid of matrix mineralization and periosteal reaction (4). Owing to substantial overlap with other common bony and vascular lesions, tissue sampling is required, which shows nests or cords of spindled endothelial cells positive for CD31, CD34, FLI1, and ERG immunophenotypic markers. However, radiologists may consider a diagnosis of EHE in the context of slow-growing, locally aggressive tumors. Treatment approaches are individualized, encompassing wide surgical excision, radiofrequency ablation, and antiangiogenic drugs (5).
Footnotes
Authors declared no funding for this work.
Disclosures of conflicts of interest: Y.S. No relevant relationships. A.G. No relevant relationships. A.R.M. No relevant relationships. K.R. No relevant relationships.
Keywords: Skeletal-Axial, Oncology, CT, Hemangioendothelioma, Osteosarcoma
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