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

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

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  • 1Department of Neurosurgery, Klinikum Nuernberg, Nuremberg, Germany.

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

PMID:
22539920
PMCID:
PMC3337738
DOI:
000336947
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