Multifocal epithelioid hemangioendothelioma derived from the spine region: case report and literature review

Case Rep Oncol. 2012 Jan;5(1):91-8. doi: 10.1159/000336947. Epub 2012 Feb 24.

Abstract

Background: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with malignant biological behavior. It arises from endothelial cells, usually within soft tissues, and can occur in almost all locations.

Case report: We report a unique case of a 25-year-old man who presented with sudden attacks of severe back pain followed by acute non-traumatic paraplegia. Emergency diagnostics revealed a pathologic fracture of the T7 vertebra with tumor tissue invasion of the spinal canal. Furthermore, multifocal metastases were found.

Results: To achieve en bloc resection, interdisciplinary surgical approaches were indicated. Despite multimodal therapy concepts, including radiotherapy and chemotherapy as well as endovascular embolization, the patient died within 8 weeks.

Conclusion: Prognosis of EHE is unpredictable and mainly determined by its location. The lesions are potentially aggressive; therefore, en bloc resection should be attempted whenever possible. However, as shown in the literature, only 15% of patients are suitable for total resection.

Keywords: Bone manifestation; Hemangioendothelioma; Interdisciplinarity; Multifocal metastasis; Multimodal treatment; Vascular spinal tumor.

Publication types

  • Case Reports