Cervical spine epithelioid hemangioendothelioma: case report

Epithelioid Hemangioendothelioma (EHE) is a rare aggressive vascular tumor and can occur in almost all locations. Spine location is rare. There are just a few cases published in the literature and the longer series included only five cases.

We report a case of a 58-year-old woman who presented with anemia and cervical pain without neurological deficit. Radiological diagnosis revealed a C2-C3 mass with vertebral artery involvement and liver metastases. Partial resection and arthrodesis followed by radiotherapy and chemotherapy were performed. Local control of the diseases was achieved but distance metastasis appears two years after surgery.

EHE of the spine is extremely rare in clinic as a primary aggressive vascular tumor. Metastatic disease is the most important factor to predict prognosis. It is not clear in the literature which is the best surgical approach in this patients and it mainly depends of the location and systemic illness. En bloc resection or expanded resection supported with adjunct radiation therapy may present with acceptable results of local control of the tumor. Systemic disease control must be achieved with chemotherapy.