MBCL-08. MOLECULAR CHARACTERIZATION OF NODULAR DESMOPLASTIC MEDULLOBLASTOMAS IN YOUNG CHILDREN TREATED ON ACNS1221. A REPORT FROM THE CHILDREN ONCOLOGY GROUP
Bibliographic record
Abstract
ACNS1221 was a single-arm multicenter trial of conventional chemotherapy for children < 4y with localized nodular desmoplastic medulloblastoma and medulloblastoma with extensive nodularity(ND/MBEN), based on a modified HIT SKK 2000 regimen excluding intraventricular methotrexate. The trial prematurely closed for an excess of relapses. Here we provide the final analysis of the clinical and molecular characterization of the cohort. Tumor samples were prospectively collected to describe the molecular profile of ND/MBEN. DNA methylation was performed using Infinium MethylationEPIC BeadChip and profiled on DKFZ molecularneuropathology2.0 classifier. DNA copy-number variants were inferred from DNA methylation arrays using Conumee. Methylation profiles were added to a founder cohort of 87 infant SHH-MB and analyzed by t-distributed stochastic neighbor embedding (t-SNE) to delineate SHH-I and SHH-II subtypes. The cohort included 26 patients(19 ND and 7 MBEN) diagnosed at a median age of 19.7 months. All tumors clustered within the SHH group and separated into 2 subtypes: SHH-I(42%) and SHH-II(58%). SHH-I patients were significantly older than SHH-II patients (p=0.009). Most MBEN were SHH-II(86%) but no significant association was found between histology and subgrouping(p=0.18). Chromosome 2 gain was exclusively described in SHH-I. Eight relapses occurred in SHH-I and 4 in SHH-II (median time of 9.8 months). The 2y PFS for SHH-I and SHH-II was respectively 27.3%(+/-13.4%) and 73.3%(+/-13.4%) (p=0.034). Age <12 mos, MBEN histology and female sex were associated with better PFS. With the ACNS1221 cohort, we prospectively validated the existence of 2 SHH subgroups in young children and the worse outcome of SHH-I subgroup.
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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 | 0.000 |
| 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 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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".