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Myxopapillary ependymoma with interval postoperative CSF seeding: A report of an unusual case
Ali M Abdu1, Sultan A Alshoabi2, Abdulbaset M Alshoaibi3
1Radiology Unit, King Saud University Medical City, Riyadh, Kingdom of Saudi Arabia.
Abstract:
Myxopapillary ependymoma (MPE) is a unique slow-growing benign (WHO grade 1) subtype of spinal cord ependymoma arising predominantly in the filum terminale. Despite its benign nature, it occasionally disseminates through the cerebrospinal fluid and metastasizes to distant sites. Here, we report an extremely rare case of MPE with interval CSF seeding and metachronous metastasis in a 47 -year-old female presented as a gradually increasing low back pain for three months with bilateral radiculopathy down to the knees. Magnetic resonance imaging (MRI) showed an intradural extramedullary spinal mass of iso-intense signal to the cord on T1 weighted-images (WIs), heterogeneous, predominantly hyperintense signal on T2WIs with homogenous enhancement after contrast administration. L2 laminectomy with gross total resection (GTR) was performed, and histopathological results confirmed the diagnosis of MPE. Adjuvant radiotherapy was administered, followed by series of MRI scans. 28 months after GTR, Lumbar MRI showed multiple tiny enhancing nodules in the cauda equina. 44 months follow-up whole spine MRI revealed multiple intradural extramedullary nodules throughout the entire spine. The largest one measures about 1.5cm opposite to T3 -T4 intervertebral disc space. The patient underwent T3 and T4 laminectomy and GTR under general anesthesia using microsurgical techniques, and the histopathological result came with the diagnosis of MPE.
Insights
This case report details a rare instance of myxopapillary ependymoma (MPE) spreading via cerebrospinal fluid (CSF) and metastasizing to distant spinal sites. The study highlights the importance of long-term surveillance for spinal cord tumors, even benign ones.
Area of Science:
- Neuro-oncology
- Spinal Cord Pathology
- Neurosurgery
Background:
- Myxopapillary ependymoma (MPE), a WHO grade 1 spinal tumor, typically arises in the filum terminale.
- Despite its benign classification, MPE can exhibit cerebrospinal fluid (CSF) dissemination and distant metastasis.
Observation:
- A 47-year-old female presented with low back pain and radiculopathy.
- Initial MRI revealed an intradural extramedullary spinal mass at L2.
- Gross total resection (GTR) and adjuvant radiotherapy were performed.
Findings:
- 28 months post-surgery, MRI showed multiple enhancing nodules in the cauda equina.
- 44 months post-surgery, whole spine MRI revealed disseminated intradural extramedullary nodules, including a 1.5cm mass at T3-T4.
- Histopathology confirmed MPE in the metastatic lesions.
Implications:
- This case underscores the potential for late recurrence and widespread seeding of MPE, even after GTR and radiotherapy.
- Highlights the necessity for vigilant, long-term neuroimaging surveillance in patients with MPE.
- Suggests a need to reconsider management strategies for MPE with metastatic potential.

