Epithelioid hemangioendothelioma of the spine: an analysis of imaging findings

Abstract

Background: Epithelioid hemangioendothelioma (EHE) is a low-grade malignant vascular neoplasm with the potential to metastasize. Primary EHE of the spine is very rare and an accurate diagnosis is crucial to treatment planning. We aim to investigate the imaging and clinical data of spinal EHE to improve the understanding of the disease. Methods: We retrospectively analyzed the imaging manifestations and clinical data of 12 cases with pathologically confirmed spinal EHE. The imaging features analyzed included number, locations, size, border, density, signal, majority of the lesions, expansile osteolysis, residual bone trabeculae, sclerotic rim, vertebral compression, enhancement. Results: Patients included 5 female and 7 male patients (mean age: 43.0 ± 19.6 years; range 15–73 years). Multiple lesions were noted in 1 case and single lesion was noted in 11 cases. The lesions were located in the thoracic, cervical, lumbar, and sacral vertebrae in 7, 3, 1, and 1 cases, respectively. They were centered in the vertebral body and posterior elements in 9 and 3 cases, respectively. Residual bone trabeculae, no sclerotic margin, and surrounding soft-tissue mass were noted in 11 cases, each, and mild expansile osteolysis and vertebral compression were noted in 10 and 6 cases, respectively. MRI was performed for 11 patients, all of whom showed isointensity on T1WI, hyperintensity or slight hyperintensity on T2WI, and hyperintensity on fat-suppressed T2WI. A marked enhancement pattern was noted in 10 cases. Conclusion: Spinal EHE tend to develop in the thoracic vertebrae. EHE should be considered when residual bone trabeculae can be seen in the bone destruction area, accompanied by pathological compression fracture, no sclerotic rim, and high signal intensity for a vascular tumor on T2WI.