AT-03ATYPICAL TERATOID RHABDOID TUMORS IN THE FIRST YEAR OF LIFE: SHOULD WE TREAT?
Bibliographic record
Abstract
BACKGROUND: While 2/3 of the patients with Atypical Teratoid Rhabdoid Tumors (ATRT) are less than 3 year at diagnosis, the literature suggest young infants present with more aggressive disease, leading dismal outcome. Very limited data exist on presentation and outcome of patients diagnosed with ATRT in the first year of life. It is unclear whether they undergo more frequently palliative management and how they fare with similar therapies used in older children. METHOD: We compared the cohort of patients ≤ 12 months from the Canadian ATRT registry to cases reported in the literature between 2000-2014. RESULTS: Twenty six patients(33.7%) included in registry were ≤ 12 months old. Review of the literature identified 120 cases. Post-operatively, 46% of our patients underwent palliative management versus 10.8 % in the literature cohort. Palliative patients were twice younger than those actively treated(3.3 versus 6.5 months) and had significantly lower rate of gross total resection(GTR). For patients actively treated, the 5 year PFS and OS were respectively 26.2% and 26.7 %. High-dose chemotherapy(HDC) was used in 42.9% of our cohort and 35.5% in the literature cohort. Canadian infants received less frequently radiotherapy(14.3% vs 44.9% p = 0.04). GTR, HDC and adjuvant radiotherapy were associated with better outcome. INI1 germline mutation was detected in 80% of the tested patients. CONCLUSION: While 1/3 of ATRT occurs within the first year of life, a large proportion of our patients received palliative therapy. A subset of selected patients benefited from HDC ± focal radiotherapy resulting in similar survival observed in older children.
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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) | 0.000 | 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".