Navigating the Unseen: A Rare Case of Intradural Extramedullary Spinal Hemangioblastoma and a Systematic Review of Surgical Challenges and Outcomes Publisher Pubmed



Esmaeilzadeh M ; Shirani M ; Rezaee H ; Abouei Mehrizi MA
Source: World Neurosurgery Published:2026

Abstract

Objective: Spinal hemangioblastomas are rare vascular lesions responsible for 1.6%–2.1% of spinal cord tumors, while intradural extramedullary (IDEM) hemangioblastomas are much rarer, especially cases not associated with Von Hippel-Lindau. The aim of this article is to review the clinical presentation, diagnosis, and surgical treatment of IDEM and to present a case of isolated cervical IDEM in a patient without Von Hippel-Lindau. Methods: A comprehensive search of databases including PubMed, Web of Science, Scopus, and Embase was performed to identify literature regarding surgical outcomes for IDEM. The current review discussed a total of 46 articles describing 62 patients treated for IDEM until 2024. We also describe here the case of a 41-year-old female with an isolated cervical IDEM presenting with neck pain, upper limb weakness, and paresthesia. Results: In this systematic review, total resection was reported in 84%. 65% of the population showed complete resolution of symptoms and 23% symptomatically improved after surgery. Complications occurred in 7%, including one death. The case presented here was treated by surgical excision via C2–C3 laminectomy followed by spinal fusion. Histopathology proved the lesion to be an IDEM. The patient had no symptoms postoperatively without recurrence on follow-up imaging. Conclusions: IDEM represents a rare benign tumor, with challenging diagnosis and surgical treatment. Advanced imaging-guided microsurgical resection and neuromonitoring enable favorable outcomes characterized by a high rate of total resection and symptom resolution. This article emphasizes on the importance of meticulous surgical planning and standardized reporting of outcomes as one way to optimize patient care. © 2026