Successful Multimodality Treatment and Long- Term Follow-Up of a Primary Spinal Epithelioid Hemangioendothelioma

Successful Multimodality Treatment and Long- Term Follow-Up of a Primary Spinal Epithelioid Hemangioendothelioma

Introduction: Epithelioid hemangioendothelioma (EHE) is an unusual vascular tumor which is rarely found in the spine. It has metastatic potential and unpredictable behavior. The choice of treatment remains controversial with the role of radiotherapy and chemotherapy still not clearly established.

Case presentation: The case of a 63-year-old female patient with a spinal EHE that presented with gradually deteriorating paraparesis and sensory deficits is reported. Radiologic work-up revealed the presence of a mid-thoracic vertebral mass which extended epidurally and into the thoracic cavity. She underwent laminectomy and removal of the epidural mass. Postoperatively, she was further treated with intravascular embolization and stereotactic radiosurgery. These resulted in significant reduction of the tumor. Histopathologic analysis confirmed the diagnosis of EHE. Seven years later, she remained symptom-free with no evidence of tumor recurrence on Magnetic Resonance Imaging.

Conclusion: Selective surgical excision combined with embolization and stereotactic radiosurgery may be effective in treating spinal EHEs obviating the need for an extensive surgery. To our knowledge, this is the first such case reported in the literature.