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Published on: November 17, 2021
Primary cerebellar glioblastomas in children: clinical presentation and management
Qiguang Wang1, Jian Cheng1, Zhang Si1
1Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.
Insights
Pediatric cerebellar glioblastomas (pcGBMs) are rare, often IDH1 wild-type, and characterized by H3K27M mutations. Routine postoperative radiotherapy and chemotherapy are recommended for improved survival in pcGBM patients.
Area of Science:
- Neuro-oncology
- Pediatric oncology
- Molecular pathology
Background:
- Pediatric cerebellar glioblastomas (pcGBMs) are rare and poorly understood brain tumors.
- Limited data exists on their clinical characteristics and molecular profiles.
Purpose of the Study:
- To analyze the clinical and molecular features of pediatric cerebellar glioblastomas.
- To evaluate the impact of treatment modalities on survival outcomes.
Main Methods:
- Retrospective analysis of 10 pediatric patients with pcGBMs treated between 2008-2019.
- Literature review of 38 additional pcGBM cases.
- Molecular analysis including IDH1, H3K27M mutations, and MGMT promoter methylation.
- Kaplan-Meier survival analysis and multivariate analysis.
Main Results:
- The study included 48 pcGBM cases with a mean age of 8.84 years.
- Increased intracranial pressure was the most common clinical sign.
- H3K27M mutations were detected in 57.1% of analyzed cases, and IDH1 mutations were absent.
- Chemotherapy and radiotherapy significantly improved overall survival (P < 0.001).
- Chemotherapy was a significant predictor of survival (Hazard Ratio = 3.264, P = 0.038).
- Mean overall survival was 12.21 months.
Conclusions:
- Pediatric cerebellar glioblastomas exhibit distinct molecular features, notably a high incidence of H3K27M mutations and IDH1 wild-type status.
- Routine postoperative radiotherapy and chemotherapy are recommended for improved survival in pcGBM patients.
- Further research into targeted therapies based on molecular profiles is warranted.
Abstract:
Pediatric cerebellar glioblastomas (pcGBMs) are rare and their characteristics remain ill-defined. We conducted a retrospective analysis of pediatric cerebellar glioblastomas who underwent surgery from 2008 to 2019 in our department. Besides, we performed a literature review of the literature data on pcGBMs. Ten children with mean age of 9.4 years were included. During the follow-up, six patients died with mean survival time of 11.7 months, four patients survived with mean follow-up of 28 months. Seven patients underwent molecular analysis, no patients detected IDH1 mutations, four patients (57.1%) had H3K27M mutations, and two patients (28.6%) had MGMT promoter methylation. The literature review identified 38 pcGBMs cases (including ours), with mean age of 8.84 ± 4.20 years (range, 1-16 years). Increased ICP was the commonest sign. Eighteen (47.4%) patients underwent GTR and fifteen (45.5%) patients received STR. Postoperative radiation (RT) was conducted in 28 patients (75.7%) and 23 patients (65.7%) received chemotherapy. During the follow-up, 25 patients died with mean survival time of 12.21 months and 11 patients survived with average follow-up of 29.3 months. Kaplan-Meier survival depicted chemotherapy (P < 0.001) or radiation (P < 0.001) had positive impact on overall survival. Multivariate analysis revealed chemotherapy was a significant predictor of survival with a hazard ratio of 3.264 (P = 0.038). Our study found mean overall survival time for pcGBMs patients was 12.21 months. PcGBMs may have distinct molecular features, with higher incidence of H3K27M mutation and were always IDH1 wild-type. We recommend the routine postoperative radiotherapy and chemotherapy in pcGBMs.
