RAS/MAPK Pathway Alteration in Pediatric Low-Grade Glioma Complicated with Ventriculo-Peritoneal Shunt Related Ascites
Bibliographic record
Abstract
Abstract Introduction: Ventriculo-Peritoneal Shunt (VPS) related ascites is a rare complication of pediatric Low Grade Gliomas (pLGG). Physiopathology of this complication is not fully understood and there is paucity of data regarding the molecular profile of pLGG gliomas complicating with ascites and the optimal management of this unusual event. Methods: International multi-institutional retrospective analysis of patients diagnosed with BRAF altered pLGG and ascites arising as a complication of VPS. Demographics, tumor characteristics, therapeutic approaches and outcomes were recorded. Results: Nineteen patients were identified. Median age at diagnosis was 14 months (IQR:7-16). Most patients (16;84.2%) presented with lesions involving the optic pathway. Mean tumor standard volume was 34.8 cm2 (range:12.5-85.4). Pilocytic Astrocytoma was the most frequent histological diagnosis (14;73.7%). Eight (42.1%) tumors harbored BRAF V600-E mutation and seven (36.8%) KIAA1549 fusion. The onset of ascites was documented at a median time of 5 months following VPS insertion. Four (21%) patients were managed with paracentesis only, 7(36.8%) required both paracentesis and shunt diversion, 7(36.8%) required only a shunt diversion and 1 (5,2%) patient was managed conservatively. Ascites was the indication to change chemotherapy regimen in 10 patients. Eight patients received targeted therapy (4 dabrafenib/4 trametinib) and 5 were radiated. Eleven patients were alive at the time of this report with a median OS of 69 months (range:3-144). Conclusions: Ascites is an early feature in the course of pLGG irrespective of alterations in the RAS/MAPK pathway with high mortality rate and should be considered as an adverse prognostic risk factor in pLGG.
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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 | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| 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".