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Published on: July 5, 2021
Intracranial ependymoma: factors affecting outcome
Maura Massimino1, Francesca R Buttarelli, Manila Antonelli
1Division of Pediatric Oncology, Istituto Nazionale dei Tumori, Milan, Italy.
Future Oncology (London, England)
|March 17, 2009
Summary
Ependymomas are brain tumors in children with suboptimal management and survival rates. Biomolecular studies reveal genetic markers like 1q25 gain and EGFR overexpression indicating poor prognosis for ependymoma patients.
Area of Science:
- Neuro-oncology
- Pediatric neurosurgery
- Molecular pathology
Background:
- Ependymomas represent a significant portion of pediatric central nervous system tumors.
- Current management strategies for intracranial ependymoma yield suboptimal outcomes, with 5-year progression-free survival rates between 30-50%.
- Prognostic factors and optimal treatment pathways remain under investigation.
Purpose of the Study:
- To review the current understanding of ependymoma biology, diagnosis, and treatment.
- To highlight recent molecular findings impacting prognosis and therapeutic strategies.
- To discuss the challenges in achieving optimal patient outcomes.
Main Methods:
- Review of recent scientific literature on ependymomas.
- Analysis of molecular profiles and their correlation with clinical outcomes.
- Evaluation of current treatment modalities and emerging therapeutic approaches.
Main Results:
- Intracranial and spinal ependymomas share molecular similarities with radial glia.
- Gain of 1q25 and EGFR overexpression are associated with poor prognosis.
- Low nucleolin expression correlates with a favorable outcome.
- Complete surgical resection is achievable in approximately 50% of cases.
Conclusions:
- Ependymoma management requires further optimization, particularly for patients with residual disease.
- Molecular markers are crucial for predicting ependymoma prognosis.
- Multimodal treatment, including surgery, radiation, and potentially chemotherapy, is essential for improving outcomes.

