Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype: case series of a new entity.
Case series
Diffuse paediatric-type high-grade glioma
H3-wildtype
IDH-wildtype
pHGG
Journal
Brain tumor pathology
ISSN: 1861-387X
Titre abrégé: Brain Tumor Pathol
Pays: Japan
ID NLM: 9716507
Informations de publication
Date de publication:
Oct 2023
Oct 2023
Historique:
received:
24
04
2023
accepted:
18
07
2023
medline:
23
10
2023
pubmed:
10
8
2023
entrez:
10
8
2023
Statut:
ppublish
Résumé
Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype (pHGG) is a rare and aggressive brain tumor characterized by a specific DNA methylation profile. It was recently introduced in the 5th World Health Organization classification of central nervous system tumors of 2021. Clinical data on this tumor is scarce. This is a case series, which presents the first clinical experience with this entity. We compiled a retrospective case series on pHGG patients treated between 2015 and 2022 at our institution. Data collected include patients' clinical course, surgical procedure, histopathology, genome-wide DNA methylation analysis, imaging and adjuvant therapy. Eight pHGG were identified, ranging in age from 8 to 71 years. On MRI tumors presented with an unspecific intensity profile, T1w hypo- to isointense and T2w hyperintense, with inhomogeneous contrast enhancement, often with rim enhancement. Three patients died of the disease, with overall survival of 19, 28 and 30 months. Four patients were alive at the time of the last follow-up, 4, 5, 6 and 79 months after the initial surgery. One patient was lost to follow-up. Findings indicate that pHGG prevalence might be underestimated in the elderly population.
Identifiants
pubmed: 37561227
doi: 10.1007/s10014-023-00468-3
pii: 10.1007/s10014-023-00468-3
pmc: PMC10575802
doi:
Substances chimiques
Isocitrate Dehydrogenase
EC 1.1.1.41
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
204-214Informations de copyright
© 2023. The Author(s).
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