Epithelioid hemangioendothelioma of the cervical spine successfully treated with subtotal resection and radiotherapy

Abstract

Epithelioid hemangioendothelioma (EHE) is a mid-grade malignant neoplasm of endothelial origin with an unpredictable clinical course and prognosis, representing <1 % of malignant bone tumors. A 63-year-old man was visited for a loss of strength in the upper right limb. MRI confirmed an expansive process in C5, PET scan did not find any other tumor localizations, and the CT-guided biopsy determined it to be an endothelial tumor. C5 corpectomy along with a C2-T2 arthrodesis was performed in a two-stage surgery, and subtotal tumor excision was obtained. Then, the patient underwent 23 radiotherapy sessions. The anatomopathological result was a low-grade EHE. At a 3-year follow-up, the patient is autonomous for ADLs with a low degree of dexterity in both hands. Preoperative CT-guided biopsy is usually non-definitive as was in the present case. Therefore, an inmunohistochemical study for the detection of factor VII antigen is crucial for definitive diagnosis. Surgical ablation should be the basis for treatment as it has been proven that “en bloc” resection reduces local recurrence. Radiotherapy has demonstrated to be helpful.