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Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype

MedGen UID:
1808288
•Concept ID:
C5669918
•
Neoplastic Process
Synonyms: Diffuse High Grade Glioma in Childhood, H3-Wildtype; Diffuse High-Grade Glioma in Childhood, H3-Wildtype; Diffuse Pediatric-Type High Grade Glioma, H3-Wildtype and IDH-Wildtype; Diffuse Pediatric-Type High-Grade Glioma, H3-Wildtype and IDH-Wildtype; diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype; Pediatric Glioblastoma, H3-Wildtype; Pediatric-Type Glioblastoma, H3-Wildtype; pHGG, H3-Wildtype
 
Monarch Initiative: MONDO:0858939

Definition

An aggressive, WHO grade 4 diffuse brain glioma usually affecting children, adolescents, or young adults. It is characterized by the absence of histone H3, IDH1, and IDH2 mutations. It includes the following subtypes, based on DNA methylation profiles: diffuse pediatric-type high grade glioma RTK1, diffuse pediatric-type high grade glioma RTK2, and diffuse pediatric-type high grade glioma MYCN. [from NCI]

Professional guidelines

PubMed

Refinement of diagnostic criteria for pediatric-type diffuse high-grade glioma, IDH- and H3-wildtype, MYCN-subtype including histopathology, TP53, MYCN and ID2 status.
Tauziède-Espariat A, Uro-Coste E, Nicaise Y, Sievers P, von Deimling A, Sahm F, Aboubakr O, Métais A, Chrétien F, Varlet P
Acta Neuropathol Commun 2023 Oct 24;11(1):170. doi: 10.1186/s40478-023-01667-x. PMID: 37875973Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Pediatric Central Nervous System Cancers, 2024

Recent clinical studies

Diagnosis

Utility of Comprehensive Genomic Profiling for Precise Diagnosis of Pediatric-Type Diffuse High-Grade Glioma.
Makino K, Otani Y, Fujii K, Ishida J, Hirano S, Suruga Y, Washio K, Nishida K, Yanai H, Tomida S, Ennishi D, Date I
Acta Med Okayama 2023 Jun;77(3):323-330. doi: 10.18926/AMO/65502. PMID: 37357634
2021 updates to the World Health Organization classification of adult-type and pediatric-type diffuse gliomas: a clinical practice review.
Thomas DL
Chin Clin Oncol 2023 Feb;12(1):7. doi: 10.21037/cco-22-120. PMID: 36922356

Prognosis

2021 updates to the World Health Organization classification of adult-type and pediatric-type diffuse gliomas: a clinical practice review.
Thomas DL
Chin Clin Oncol 2023 Feb;12(1):7. doi: 10.21037/cco-22-120. PMID: 36922356

Clinical prediction guides

Utility of Comprehensive Genomic Profiling for Precise Diagnosis of Pediatric-Type Diffuse High-Grade Glioma.
Makino K, Otani Y, Fujii K, Ishida J, Hirano S, Suruga Y, Washio K, Nishida K, Yanai H, Tomida S, Ennishi D, Date I
Acta Med Okayama 2023 Jun;77(3):323-330. doi: 10.18926/AMO/65502. PMID: 37357634

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Pediatric Central Nervous System Cancers, 2024

    Consumer resources

    Reviews

    Related information

    • ClinVar
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