High-Grade Gliomas in Children-A Multi-Institutional Polish Study

Simple Summary High-grade gliomas constitute less than 5% of pediatric brain tumors. Due to the rarity of such a diagnosis, the lack of consensus about the best therapeutic approach, and the difficulty in conducting prospective trials; a retrospective multi-institutional analysis, such as the one presented in this article, is needed. We carried out the survival analysis of children diagnosed and treated with high-grade gliomas in seven major polish institutions. The assessment of the outcome of 82 consecutive patients with grade III and grade IV tumors was performed and showed a 5-year overall survival of only 30%. The extent of resection, immediate temozolomide-based chemotherapy, and radical radiotherapy were found as factors positively influencing survival. Due to the rarity of high-grade gliomas (HGG) in children, data on this topic are scarce. The study aimed to investigate the long-term results of treatment of children with HGG and to identify factors related to better survival. We performed a retrospective analysis of patients treated for HGG who had the main tumor located outside the brainstem. The evaluation of factors that correlated with better survival was performed with the Cox proportional-hazard model. Survival was estimated with the Kaplan-Meier method. The study group consisted of 82 consecutive patients. All of them underwent surgery as primary treatment. Chemotherapy was applied in 93% of children with one third treated with temozolomide. After or during the systemic treatment, 79% of them received radiotherapy with a median dose of 54 Gy. Median follow-up was 122 months, and during that time, 59 patients died. One-, 2-, 5-, and 10-year overall survival was 78%, 48%, 30% and 17%, respectively. Patients with radical (R0) resection and temozolomide-based chemotherapy had better overall survival. Progression-free survival was better in patients after R0 resection and radical radiotherapy. The best outcome in HGG patients was observed in patients after R0 resection with immediate postoperative temozolomide-based chemotherapy and radical radiotherapy.

Automated summary

A retrospective analysis of 82 pediatric patients treated for high-grade gliomas showed a 5-year overall survival rate of only 30%. Factors such as extent of resection, chemotherapy, and radiotherapy were found to positively influence survival. High-grade gliomas in children remain rare and data on long-term outcomes are limited.