Disseminated pediatric low-grade glioma and hydrocephalus: a multinational consortium analysis of incidence and mortality
Bibliographic record
Abstract
OBJECTIVE: Disseminated pediatric low-grade gliomas (DPLGGs) are a rare subtype of an otherwise common tumor, characterized by leptomeningeal dissemination, with microtumors spreading throughout the CNS. The impact of this dissemination on CSF dynamics remains unexplored. The authors describe the occurrence, treatment, and impact of hydrocephalus on functional outcomes and survival in patients with DPLGG. METHODS: This study was a post hoc analysis of a multicenter international cohort study that identified 261 children diagnosed with DPLGG from 30 sites across 13 countries from 1988 to 2025. Demographic, histopathological, radiographic, dissemination pattern, hydrocephalus treatment, and functional outcome variables were collected. The primary outcomes were survival, CSF diversion failure, and time to failure (TTF). Group comparisons were conducted using independent-sample t-tests and chi-square tests. Multivariate logistic regression was performed examining predictors of hydrocephalus in DPLGG. Kaplan-Meier analysis was used to assess survival and TTF. RESULTS: One hundred forty-five (55.6%) patients developed hydrocephalus and required CSF diversion. Histopathological diagnosis differed between the groups (p = 0.02). Patients with diffuse leptomeningeal glioneuronal tumor had a 41% (OR 1.408, 95% CI 0.413-4.809) increase in odds of developing hydrocephalus relative to other histopathologies. Tumor dissemination pattern did not significantly impact hydrocephalus development (p = 0.381). There was, however, a significant association between the timing of hydrocephalus diagnosis and CSF diversion and dissemination pattern (p < 0.001). For initial CSF diversion, 124 patients (87.9%) received ventriculoperitoneal shunting (VPS) while the remaining patients underwent either endoscopic third ventriculostomy (ETV) (9.2%) or septostomy (2.9%). Fifty-nine (43.1%) patients who underwent CSF diversion required hydrocephalus reintervention at an overall median TTF of 4.96 months (IQR 0.8-22.4) months. TTF by CSF diversion modality showed no significant difference by Kaplan-Meier analysis (log-rank test, p = 0.90). There was no difference in overall survival (log-rank test, p = 0.95) between the hydrocephalus and nonhydrocephalus groups. However, hydrocephalus was associated with academic difficulties (p = 0.02) and concurrent endocrine disorders (p = 0.03). CONCLUSIONS: This study represents the largest and most comprehensive cohort of patients with DPLGG to date. While histopathology and tumor location were associated with hydrocephalus in this cohort, the dissemination pattern was not directly associated with hydrocephalus incidence but rather the timing of hydrocephalus diagnosis. Hydrocephalus does not impact survival in patients with DPLGG; however, it is associated with worse functional outcomes.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".