• Shannan Bialek, Lance M Villeneuve, Andrew Bauer, Minh Phan, Andrea Johnston, Caple Spence, and Christopher S Graffeo.
• Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK.
• World Neurosurg. 2024 Aug 1; 188: 9911099-110.

BackgroundEpithelioid hemangioendothelioma (EHE) is a rare vascular tumor predominantly arising in soft tissue. We report a rare case of thoracic spinal EHE with pulmonary metastasis.MethodsCase report and systematic review of spinal EHE.ResultsA 36-year-old man presented with bilateral lower extremity weakness, progressive paresthesia, and urinary incontinence. He underwent open surgical resection of the tumor and decompression of the spinal cord, with subsequent improvement in neurologic function. Systematic review identified 84 cases of spinal EHE, 73 of which were primary, and 14 of which developed extra-spinal metastases.ConclusionEHE is an exceedingly rare tumor that may present with a wide swath of clinical symptoms. At present, no guidelines or formal treatment recommendations have been established. Surgical debulking has demonstrated efficacy as a front-line treatment, particularly in the setting of compressive neurologic dysfunction; data regarding adjuvant chemoradiation are less consistently reported, mandating further study.Copyright © 2024 Elsevier Inc. All rights reserved.

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