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Gangliogliomas in the pediatric population
Arnold R Quiroz Tejada1, Pablo Miranda-Lloret2, Margarita Llavador Ros3
1Department of neurological surgery, HUiP La Fe, Valencia, Spain. arnold81492@hotmail.com.
Summary
Gangliogliomas are rare pediatric brain tumors. While complete resection offers cures, some residual tumors progress, suggesting BRAF inhibitors as a potential treatment for these challenging cases.
Area of Science:
- Pediatric neuro-oncology
- Tumor pathology
- Neurosurgery
Background:
- Gangliogliomas are mixed glial and neural cell tumors, comprising 5% of pediatric CNS tumors.
- These slow-growing tumors are often curable with complete surgical resection.
- Tumor progression after incomplete resection necessitates further treatment strategies.
Purpose of the Study:
- To analyze the clinical characteristics and outcomes of pediatric gangliogliomas.
- To evaluate the efficacy of gross total tumor resection (GTR) in pediatric gangliogliomas.
- To identify potential therapeutic targets for progressing gangliogliomas.
Main Methods:
- Retrospective study of pediatric patients with ganglioglioma.
- Data collected from medical records between 2001 and 2020.
- Analysis of surgical outcomes, clinical presentation, and molecular markers.
Main Results:
- 17 pediatric patients included; median age 6.7 years, median follow-up 60 months.
- Epileptic seizures were the most common presentation (41.1%).
- Gross total tumor resection (GTR) achieved in 47% of cases; 33% of those without GTR showed progression. BRAF V600E mutation found in 44.4%.
Conclusions:
- Gangliogliomas, typically Grade I, pose surgical challenges in pediatric patients.
- While many residual tumors stabilize, some progress, indicating a need for adjuvant therapies.
- BRAF inhibitors represent a promising treatment option for progressive gangliogliomas.
