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Updated: May 10, 2026
Intraoperative Ultrasound in Spinal Surgery
Published on: August 17, 2022
Intradural extramedullary spinal ependymoma: a case report of malignant transformation occurring
Takashi Moriwaki1, Koichi Iwatsuki, Yu-Ichiro Ohnishi
1Department of Neurosurgery, Osaka University Graduate School of Medicine, Suita, Japan.
Abstract:
Intradural extramedullary spinal ependymomas are extremely rare. Herein, we describe a lesion-type spinal ependymoma that followed a malignant course, and discuss its clinical presentation, etiopathogenesis, and treatment. We present a patient who was diagnosed with an intradural extramedullary spinal tumor at T4-T6. The patient underwent gross total resection of the tumor without damage to the spinal cord. Histological examination, classified the lesion as a World Health Organization (WHO)-grade 2 ependymoma. One and a half years later, magnetic resonance imaging detected a recurring tumor at T4-T5. The tumor was removed and classified as a WHO-grade 3 anaplastic ependymoma. The patient was started on a course of regional spinal cord radiotherapy. The patient achieved tumoral control and clinical stabilization after the recurrence. We must consider the differential diagnosis of intradural extramedullary spinal tumors. The best treatment for this lesion is gross total resection and adjunctive radiotherapy is necessary in cases of malignant-change.
Insights
Intradural extramedullary spinal ependymomas are rare tumors. This case highlights a World Health Organization (WHO) grade 2 ependymoma that progressed to grade 3, emphasizing the need for aggressive treatment.
Area of Science:
- Neurosurgery
- Oncology
- Spinal Cord Pathology
Background:
- Intradural extramedullary spinal ependymomas are exceptionally rare.
- These tumors originate within the dura but outside the spinal cord itself.
- Their malignant potential and clinical course require careful consideration.
Purpose of the Study:
- To describe a rare case of a lesion-type spinal ependymoma with malignant progression.
- To discuss the clinical presentation, etiopathogenesis, and optimal treatment strategies.
- To emphasize the importance of differential diagnosis for intradural extramedullary spinal tumors.
Main Methods:
- Case report of a patient diagnosed with an intradural extramedullary spinal tumor.
- Surgical gross total resection of the tumor.
- Histopathological classification according to World Health Organization (WHO) grading.
- Magnetic resonance imaging (MRI) for recurrence detection.
- Post-operative radiotherapy.
Main Results:
- Initial diagnosis: WHO grade 2 ependymoma at T4-T6.
- Recurrence detected 1.5 years later at T4-T5, classified as WHO grade 3 anaplastic ependymoma.
- Successful gross total resection and subsequent radiotherapy led to tumoral control and clinical stabilization.
Conclusions:
- Spinal ependymomas can exhibit malignant progression, necessitating vigilant follow-up.
- Gross total resection is the primary treatment modality.
- Adjuvant radiotherapy is crucial for managing malignant-grade spinal ependymomas to achieve tumoral control.
