HGG-14. RAS PATHWAY MEDIATED REGULATION OF HISTONE METHYLTRANSFERASE AND TUMOR SUPPRESSOR SETD2 IN PEDIATRIC HIGH GRADE GLIOMA

Abstract BACKGROUND Pediatric High-Grade Gliomas (pHGG) are a significant cause of cancer-associated deaths in children. Hemispheric pHGG have frequent loss of the NF1 tumor suppressor (15%) causing aberrant RAS pathway activation. Intriguingly, loss of SETD2, a H3K36 methyltransferase, significantl...

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Bibliographic Details
Published in:Neuro-oncology (Charlottesville, Va.) Va.), 2023-06, Vol.25 (Supplement_1), p.i42-i42
Main Authors: Liou, Angela, Moran, Deborah, Dougherty, Jacquelyn, Harvey, Kyra, Brosius, Stephanie, Labella, Kay, De Raedt, Thomas
Format: Article
Language:English
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Final Category: High Grade Glioma/Gliomatosis Cerebri - HGG
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Summary:Abstract BACKGROUND Pediatric High-Grade Gliomas (pHGG) are a significant cause of cancer-associated deaths in children. Hemispheric pHGG have frequent loss of the NF1 tumor suppressor (15%) causing aberrant RAS pathway activation. Intriguingly, loss of SETD2, a H3K36 methyltransferase, significantly co-occurs with loss of NF1. We found that nearly 80% SETD2-mutant pHGG have inactive NF1 (Pedcbio, 18/23 cases, 78%; odds-ratio 25; FDR adjusted P-value
ISSN:1522-8517
1523-5866
DOI:10.1093/neuonc/noad073.163