Multifocal Epithelioid Hemangioendothelioma Derived from the Spine Region: Case Report and Literature Review |
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| Authors: | G. Kerry O. Marx D. Kraus M. Vogel A. Kaiser C. Ruedinger H.H. Steiner |
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| Affiliation: | aDepartment of Neurosurgery, Klinikum Nuernberg, Nuremberg, Germany;bGeneral, Visceral and Thoracic Surgery, Klinikum Nuernberg, Nuremberg, Germany;cRadiology, Klinikum Nuernberg, Nuremberg, Germany;dPathology, Klinikum Nuernberg, Nuremberg, Germany |
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| Abstract: | BackgroundEpithelioid 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 ReportWe 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.ResultsTo 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.ConclusionPrognosis 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.Key words: Hemangioendothelioma, Vascular spinal tumor, Bone manifestation, Multifocal metastasis, Interdisciplinarity, Multimodal treatment |
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