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Isolated myxopapillary ependymoma of the fourth ventricle: illustrative case
Alemu A Mldie1, Abat S Baleh2, Henok T Molla2
1Department of Surgery, Neurosurgery Unit, Jimma University, Jimma, Ethiopia.
Background:
A myxopapillary ependymoma (MPE) is a distinctive ependymoma variant that arises exclusively in the conus medullaris or the filum terminale. There are few case reports of intracranial occurrences, with even rarer occurrences in the fourth ventricle.
Observations:
A 31-year-old female patient presented with difficulty in maintaining her balance and headaches of 3 months' duration. Brain magnetic resonance imaging showed a fourth ventricle mass with obstructive hydrocephalus. Surgical removal of the tumor was performed. The biopsy of the tumor revealed the histological feature of MPE, World Health Organization grade 2. To the authors' knowledge, this is the fourteenth reported case of primary intracranial MPE and the fifth reported case of MPE originating from the fourth ventricle.
Lessons:
MPEs are known for their notorious adhesion and infiltration of adjacent neural tissue; therefore, the clinical outcome of fourth ventricle MPEs depends on their relationship with the floor of the fourth ventricle. https://thejns.org/doi/10.3171/CASE24571.
Insights
This case report details a rare intracranial myxopapillary ependymoma (MPE) in the fourth ventricle. Surgical removal was successful, highlighting the importance of tumor location for patient outcomes.
Area of Science:
- Neuro-oncology
- Neurosurgery
- Pathology
Background:
- Myxopapillary ependymomas (MPEs) are rare tumors typically found in the spinal cord.
- Intracranial MPEs are exceptionally uncommon, with fourth ventricle origin being particularly rare.
Purpose of the Study:
- To report a rare case of a 31-year-old female with a fourth ventricle myxopapillary ependymoma.
- To discuss the clinical presentation, surgical management, and pathological findings of this rare intracranial tumor.
Main Methods:
- A case study of a 31-year-old female patient presenting with balance issues and headaches.
- Brain MRI revealed a fourth ventricle mass causing obstructive hydrocephalus.
- Surgical tumor resection followed by histopathological analysis confirmed MPE, WHO grade 2.
Main Results:
- The patient presented with symptoms of increased intracranial pressure and neurological deficits.
- Histopathology confirmed the diagnosis of myxopapillary ependymoma, WHO grade 2.
- This represents the 14th reported case of primary intracranial MPE and the 5th originating in the fourth ventricle.
Conclusions:
- Fourth ventricle myxopapillary ependymomas are rare but require surgical intervention.
- Tumor adhesion and infiltration necessitate careful surgical planning.
- Clinical outcomes are influenced by the tumor's relationship with the floor of the fourth ventricle.

