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Navigating the Unseen: A Rare Case of Intradural Extramedullary Spinal Hemangioblastoma and a Systematic Review of
Mahla Esmaeilzadeh1, Mohammad Shirani2, Hamid Rezaee3
1Sinus and Surgical Endoscopic Research Center, Mashhad University of Medical Sciences, Mashhad, Iran; Neurosurgical Department, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
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.
