Challenges in Management of Epithelioid Hemangioendothelioma of the Spine: The Report of a Rare Cause of Cauda Equina Syndrome

Abstract

Introduction: Epitheliod hemangioendothelioma (EHE) is a rare spinal neoplasm with vascular provenance, which can present as a primary lesion or metastatic lesion with the secondary involvement of the liver, lung, and long bones. EHE can occur all over the spine from the craniovertebral junction to the lumbosacral area. Usual presenting symptoms are back pain, pathologic fractures, myelopathy, and cauda equina syndrome. Diagnosis and management can be difficult, often requiring coordination between multiple disciplines for selective spinal angiography, surgical resection, the establishment of pathologic diagnosis, and adjuvant treatment, if necessary. There are no widely accepted guidelines for the management of EHE. Case Presentation: We report a 43-year-old male as a case of primary unifocal hemangioendothelioma with two unique features compared to previous reports: first, the presentation with acute cauda equine syndrome and second, the involvement of posterior elements, which made it very difficult to differentiate from aneurysmal bone cyst (ABC). Conclusions: We review the existing literature on spinal EHE, present management challenges via case review, and propose a management guideline for neurosurgeons, interventional radiologists, pathologists, and radiation oncologists to streamline the diagnosis and management of EHE of the spine.