HGG-21. A Systematic Study of Molecular Diagnosis, Treatment, Prognostic Factors and Survival Outcome in Patients with Infant-Type Hemispheric Glioma (IHG) Patients: A Meta-analysis of 164 patients
Bibliographic record
Abstract
Abstract Infant-type hemispheric glioma (IHG) is newly recognized in the fifth edition of the World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS). Diagnosed during infancy, these large hemorrhagic gliomas commonly harbor structural rearrangements in receptor tyrosine kinases (RTKs) and have a distinct methylation profile. Due to the novelty and rarity of IHG, optimal treatment and factors determining clinical outcomes are yet to be established. Here, we curated 164 IHG cases; 155 identified by methodical literature search and 9 new cases collected from collaborators. All tumors were hemispheric in location, were diagnosed at a median age of 3 (0-52) months, and were frequently (95%) non-metastatic. 95% cases reported fusions in RTKs (ALK [45%], NTRK1/2/3[21.5%], ROS1[19.5%], MET [8.5%] and ABL2 [0.5%]). 64%, 20%, and 8% of patients were treated with surgery + adjuvant chemotherapy, surgery-only and surgery + targeted therapy (TT), respectively. 3-year event free survival (EFS) and overall survival (OS) was 47.9% [39.1-58.7] and 79.8% [72.4-88.0]. Only 5 patients received radiation during their treatment course. Twenty-three patients succumbed to disease, of which tumor progression (52%) and intra-cranial hemorrhage (26%) were the most common causes of death. In multivariate analysis, presence of residual tumor significantly increased the risk of death (p < 0.05); 3-year EFS for patients with versus without residual disease was 33.5% versus 54.8% (p < 0.05);3-year OS was 62.8% versus 94.0% (p < 0.05). Patients treated with surgery-only also showed comparatively worse 3-Y- EFS 31.2% (p < 0.05). In conclusion, IHG is a fusion driven tumor, complete surgical resection is associated with a better prognosis and, paradoxically, children die from intracranial hemorrhage (likely surgically induced trauma). Furthermore, we identified a sizeable gap between EFS and OS. Together, these findings show that patients can survive progression and suggest that optimal primary therapy for IHG has not been established.
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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 | 0.003 | 0.004 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.004 | 0.011 |
| Bibliometrics | 0.002 | 0.003 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| 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".