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Record W7135049491 · doi:10.1093/neuped/wuaf001.156

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

2025· article· en· W7135049491 on OpenAlex
Aditi Bagchi, Lara Chavaz, Sandeep Dhanda, Fabienne Toutain, Stefan M Pfister, Dominik Strum, Torsten Pietsch, Gerrit H. Gielen, Andreas Waha, Mathew Clarke, Congyu Lu, Martin Benesch, Thomas Perwein, Gunther Nussbaumer, CM Kramm, Maura Massimino, Veronica Biassoni, Maria Vinci, Angela Mastronuzzi, Dannis van Vuurden, Sophie E. M. Veldhuijzen van Zanten, Alan Mackay, Chris Jones, David T W Jones, Ana S Guerreiro Stucklin, Uri Tabori, Cynthia Hawkins, Scott Ryall, Andrés Morales La Madrid, Álvaro Lassaletta, Simon Bailey, Darren R Hargrave, Jason Chiang, Moatasem El-Ayadi, Bruna Minniti Mançano, Rui Reis, Christian Hagel, Hamza Gorzi, Nicolas Silvestrini, Ahmed Gilani, L. I. Papusha, X. H. Zhou, Amar Gajjar, Giles W Robinson, Andre O vonBuren

Why this work is in the frame

A frame that forgets how it found something cannot be audited. These are the routes that admitted this work.

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.

Bibliographic record

VenueNeuro-Oncology Pediatrics · 2025
Typearticle
Languageen
FieldMedicine
TopicGlioma Diagnosis and Treatment
Canadian institutionsHospital for Sick ChildrenInstitute of Cancer Research
Fundersnot available
KeywordsDiseaseGliomaMultivariate analysisOverall survivalNoveltyAdjuvant therapyTyrosine kinaseReceptor tyrosine kinase

Abstract

fetched live from OpenAlex

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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Full frame distilled prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.002
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesMeta-epidemiology (narrow)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.174
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.002
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0040.000
Bibliometrics0.0010.004
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

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.

Opus teacher head0.027
GPT teacher head0.298
Teacher spread0.270 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it