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Supratentorial ependymomas in children: Analysis of nine cases
George A Alexiou1, Maria Moschovi, Kalliopi Stefanaki
1Department of Neurosurgery, Children's Hospital "Agia Sofia", Athens, Greece.
Insights
Pediatric supratentorial ependymomas show high expression of proliferation markers like Ki-67/MIB-1 and proteins such as p-53, bcl-2, p-27, and EGFR. Further multi-institutional studies are needed to clarify their prognostic significance.
Area of Science:
- Pediatric neuro-oncology
- Molecular pathology of brain tumors
Background:
- Intracranial ependymomas are the third most common pediatric primary brain tumor.
- Understanding molecular markers is crucial for prognosis.
Purpose of the Study:
- Investigate the expression of p-53, p-27, bcl-2, EGFR, and neuronal markers.
- Correlate marker expression with Ki-67/MIB-1 proliferation index and patient prognosis.
Main Methods:
- Retrospective analysis of nine pediatric supratentorial ependymomas.
- Surgical treatment with assessment of resection extent (gross total vs. subtotal) via MRI.
- WHO classification of ependymal tumors.
- Immunohistochemical analysis for Ki-67/MIB-1, p-53, p-27, bcl-2, EGFR, and neuronal markers.
Main Results:
- Headache, seizures, and papilledema were common symptoms/signs.
- All tumors were anaplastic with Ki-67/MIB-1 expression in 20-40% of nuclei.
- Increased expression of p-53, bcl-2, p-27, and EGFR was observed.
- Neuronal markers were expressed in three cases.
- After a mean follow-up of 32.1 months, 8/9 children were alive, with 5/9 experiencing tumor recurrence.
Conclusions:
- Complete surgical excision is the primary surgical goal.
- The prognostic value of Ki-67, p-53, p-27, bcl-2, EGFR, and neuronal markers requires further investigation in larger, multi-institutional studies due to tumor rarity.
Background:
Intracranial ependymomas are the third most common primary brain tumor in children. In the present study, we set out to investigate the expression of p-53, p-27, bcl-2, epidermal growth factor receptor (EGFR) and of neuronal markers in pediatric supratentorial ependymomas, in correlation with Ki-67/MIB-1 proliferation index and prognosis.
Materials And Methods:
Nine children with supratentorial ependymomas that were treated surgically in our institute over the last seven years were identified and included in the study. The extent of resection was classified as gross total and subtotal, and was determined by MRI scans. The ependymal tumors were classified according to WHO classification.
Results:
Headache and seizures were the most common presenting symptoms and papilledema the most common sign. In seven cases, gross total excision was performed, and in two cases, the resection was subtotal. All ependymomas were anaplastic. Ki-67/MIB-1 was detected in 20-40% of the nuclei in all tumors. There was also increased expression of p-53, bcl-2, p-27, and EGFR. There was expression of neuronal markers in three cases. After a mean follow-up period of 32.1 months (range 16-74 months), eight children were alive. Five children suffered from tumor recurrence.
Conclusions:
Complete surgical excision should be the goal of surgery. The prognostic role of Ki-67, p-53, p-27, bcl-2, EGFR, and neuronal markers expression needs to be determined in multi-institutional studies due to tumor's rarity.
