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Pulvinar Locus is Highly Relevant to Patients' Outcomes in Surgically Resected Thalamic Gliomas in Children
Kentaro Chiba1, Yasuo Aihara1, Kenta Masui2
1Department of Neurosurgery, Tokyo Women's Medical University, Tokyo, Japan.
Insights
Pediatric thalamic gliomas in the pulvinar region are linked to aggressive H3 K27M-mutant tumors and poor prognosis. Tumor location significantly impacts survival, necessitating careful surgical planning for these challenging pediatric brain tumors.
Area of Science:
- Pediatric neuro-oncology
- Neurosurgery
- Cancer genetics
Background:
- Thalamic gliomas in children present surgical challenges due to their critical location.
- Prognosis is influenced by histology, extent of resection, and surgical planning.
Purpose of the Study:
- To investigate the prognostic significance of tumor location in pediatric thalamic gliomas.
- To analyze the association between tumor location, histology, and molecular markers like H3 K27M mutation.
Main Methods:
- Retrospective analysis of 10 pediatric patients with thalamic gliomas undergoing surgical resection.
- Histopathological classification using WHO 2016 criteria, including H3 K27M mutation status.
- Correlation of tumor location (especially pulvinar region) with outcomes and molecular features.
Main Results:
- Seven high-grade and three low-grade gliomas were identified.
- Low-grade gliomas showed good progression-free survival (58.3 months).
- High-grade gliomas had a poor overall survival (28.1 months), with pulvinar location strongly predicting poor prognosis (P=0.0018) and association with H3 K27M mutation (P=0.0036).
- Pulvinar tumors frequently disseminated early, leading to fatal outcomes.
Conclusions:
- Pulvinar location is a critical factor in pediatric thalamic gliomas, associated with high malignancy, H3 K27M mutation, and early dissemination.
- Tumor location within the thalamus, particularly the pulvinar, dictates a distinct biological profile and prognosis.
- Location is a key consideration for treatment planning in pediatric thalamic gliomas.
Objective:
Thalamic gliomas in children are less suitable for surgical resection because of their location. In cases of unavoidable resection, careful surgical planning in addition to histology and extent of resection affects prognosis.
Methods:
A cohort of 10 pediatric patients with thalamic glioma underwent surgical resection at our department. The predominant location of tumor origins in the thalamus was defined in imaging studies. Histopathology was determined (retrospectively in a subset) according to the World Health Organization classification 2016, including the newly established type of "diffuse midline glioma, H3 K27M-mutant."
Results:
Three low-grade gliomas (grade I/II) and 7 high-grade gliomas (grade III/IV) were identified. The mean follow-up period was 49.8 months. All 3 low-grade gliomas did not recur (progression-free survival, 58.3 months). Six of 7 high-grade gliomas recurred, and the patients died of the primary disease (overall survival, 28.1 months). Poor outcomes, especially when located at the pulvinar region, were noticeable, with strong predictive power for poor prognosis (P = 0.0018). The presence of H3 K27M mutation and pulvinar location were closely associated (P = 0.0036). Four of 5 patients with pulvinar region tumors developed dissemination and died of the primary disease.
Conclusions:
Pulvinar location is specifically associated with a high rate of malignancy in histology, the presence of H3 K27M mutation, and dissemination at an early disease stage. This association suggests that a distinct biological profile affects prognosis depending on location within the thalamus, especially the pulvinar. We report that tumor location is highly relevant to prognosis and should be taken into consideration when planning treatment.
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