HG-75CLINICAL, RADIOLOGICAL, AND HISTO-GENETIC CHARACTERISTICS OF LONG-TERM SURVIVORS OF DIFFUSE INTRINSIC PONTINE GLIOMA: A COLLABORATIVE REPORT FROM THE INTERNATIONAL AND SIOP-E DIPG REGISTRIES
Bibliographic record
Abstract
BACKGROUND: Diffuse intrinsic pontine glioma (DIPG) is a childhood tumor with median survival of < 1 year. The International and SIOP-E DIPG Registries were established to better understand this rare disease. METHODS: Clinical, radiological, and histo-molecular, proteomics data were abstracted from registry databases and included patients from North America, Australia, Germany, Austria, Switzerland, Netherlands, Italy, United Kingdom, and Croatia. RESULTS: Among 909 patients with centrally radiographically confirmed DIPG, 53 were excluded (40 alive < 2 years, 13 survival unknown). Of 856 remaining, 78 (9%) were long-term survivors (LTS; overall survival ≥ 2 years). Median age at diagnosis was 6.7 years for both LTS (1.9-27.7 years) and short-term survivors (STS) (0.1-26.5 years). LTS more commonly presented at < 3 years (p = 0.049) and with longer symptom duration (p = 0.001). Cranial nerve palsies were more common in STS (p = 0.003), as was enhancement (p = 0.004) or necrosis (p = 0.04) on diagnostic imaging. LTS were biopsied more often (31% vs 19%), though histological grade was not significantly different between groups. Use of radiation was similar between groups; however, LTS more often received chemotherapy at diagnosis (p = 0.02). Of 27 patients with molecular data, 22 harbored histone mutations (2 LTS, 20 STS). Genomic and proteomic data for 55 (6 LTS, 49 STS) and 22 additional patients, respectively, are under analysis. CONCLUSION: In the largest DIPG cohort reported, to date, preliminary data suggest that LTS have younger age, longer symptom duration, and less radiographic evidence of enhancement or necrosis at diagnosis. They may, however, harbor similar genetic aberrations as STS patients.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".