Intramedullary Ependymoma of the Cervicomedullary Junction: Gross Total Resection and Surgical Nuances in an Anatomically Rare Location
TL;DR
This case suggests that gross total resection of cervicomedullary ependymomas under intraoperative neuromonitoring is achievable and can result in excellent neurological outcomes, highlighting the importance of precise microsurgical techniques and intraoperative monitoring in managing these challenging tumors.
Abstract
Background Intramedullary ependymomas are rare, slow-growing tumors (WHO grade II in most cases) arising from the ependymal cells of the spinal cord’s central canal. In this case, histopathological examination confirmed a tanycytic ependymoma (WHO grade II). Although they have low potential for infiltration and seeding, and complete surgical excision is generally curative, the complex anatomy of the cervicomedullary region makes total resection particularly challenging. Case Presentation We present the case of a 34-year-old woman who presented with subacute quadriparesis (McCormick grade III, MRC 3–4/5), diminished gag reflex, hyperreflexia, and spastic gait. Magnetic resonance imaging (MRI) suggested the diagnosis of a cervicomedullary junction (CMJ) ependymoma and definitive diagnosis was established histopathologically. The patient underwent successful microscopic total resection via a posterior midline approach, including suboccipital craniectomy, C1 and C2 laminectomy, and midline myelotomy, all performed under intraoperative neuromonitoring. At one-year follow-up, the patient demonstrated improvement to McCormick grade I with MRC grade 5/5 in all limbs. Postoperative MRI confirmed complete resection without evidence of local recurrence. Conclusion Neurological recovery was assessed using the McCormick Scale and Medical Research Council (MRC) muscle strength grading. The patient improved from a preoperative McCormick grade III (significant motor weakness requiring assistance for ambulation) to grade I (normal neurological function) at one-year follow-up. MRC muscle strength improved from 3–4/5 in all limbs preoperatively to 5/5 in all limbs postoperatively. Postoperative MRI at one-year follow-up confirmed complete resection without evidence of local recurrence. This case suggests that gross total resection of cervicomedullary ependymomas under intraoperative neuromonitoring is achievable and can result in excellent neurological outcomes, highlighting the importance of precise microsurgical techniques and intraoperative monitoring in managing these challenging tumors.