Clinical Management of Intracranial Ependymomas
Summary
Intracranial ependymomas are glial neoplasms arising from the ependymal lining of the ventricular system and the central canal, accounting for approximately 2‒3 % of primary central nervous system tumours. They span a spectrum from benign subependymomas (WHO grade I) and classic ependymomas (WHO grade II) to anaplastic ependymomas (WHO grade III). Clinical management hinges on maximal safe surgical resection, with gross total resection remaining the cornerstone of therapy. Postoperative radiotherapy is generally recommended for anaplastic lesions and for subtotal resections of higher‐grade tumours to reduce recurrence risk. Advances in preoperative imaging, including diffusion and perfusion MRI, have improved delineation of tumour borders and guided neurosurgical planning. Molecular profiling has revealed distinct subgroups, such as RELA fusion-positive supratentorial ependymomas, informing prognosis and potential targeted treatments. Longitudinal management employs regular neuroimaging surveillance, with formal assessment of progression-free and overall survival metrics. Multidisciplinary coordination among neurosurgeons, neuro-oncologists and radiation specialists is essential to optimise functional outcome and quality of life, particularly in paediatric populations where treatment‐related sequelae require careful mitigation.
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Clinical Management of Intracranial Ependymomas publication trend
The graph below shows the total number of articles in clinical management of intracranial ependymomas across all publications each year (not limited to Nature Index journals).
Technical terms
Gross total resection (GTR): Surgical removal of all visible tumour tissue.
WHO grade: World Health Organization classification indicating tumour malignancy and aggressiveness.
Progression-free survival (PFS): Interval from treatment initiation to tumour progression or recurrence.
Overall survival (OS): Duration from diagnosis or treatment start to death from any cause.
RELA fusion: Oncogenic gene fusion characteristic of a supratentorial ependymoma subgroup that influences prognosis.
References
- Surgical management and long-term outcome of intracranial subependymoma. Acta Neurochirurgica (2018).
- The Survival and Prognostic Factors of Supratentorial Cortical Ependymomas: A Retrospective Cohort Study and Literature-Based Analysis. Frontiers in Oncology (2020).
- Intraventricular Subependymoma With Obstructive Hydrocephalus: A Case Report and Literature Review. Cureus (2024).
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