Journal Article

DKFZ-2023-02322

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Pediatric low-grade glioma: State-of-the-art and ongoing challenges.

Fangusaro, J. ; Jones, D. T.DKFZ* ; Packer, R. J. ; Gutmann, D. H. ; Milde, T.DKFZ* ; Witt, O.DKFZ* ; Mueller, S. ; J Fisher, M. ; Hansford, J. R. ; Tabori, U. ; Hargrave, D. ; Bandopadhayay, P.

2024
Oxford Univ. Press Oxford

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Please use a persistent id in citations: doi:10.1093/neuonc/noad195

Abstract: The most common childhood central nervous system (CNS) tumor is pediatric low-grade glioma (pLGG), representing 30%-40% of all CNS tumors in children. Although there is high associated morbidity, tumor-related mortality is relatively rare. pLGG is now conceptualized as a chronic disease, underscoring the importance of functional outcomes and quality-of-life measures. A wealth of data has emerged about these tumors, including a better understanding of their natural history and their molecular drivers, paving the way for the use of targeted inhibitors. While these treatments have heralded tremendous promise, challenges remain about how to best optimize their use, and the long-term toxicities associated with these inhibitors remain unknown. The International Pediatric Low-Grade Glioma Coalition (iPLGGc) is a global group of physicians and scientists with expertise in pLGG focused on addressing key pLGG issues. Here, the iPLGGc provides an overview of the current state-of-the-art in pLGG, including epidemiology, histology, molecular landscape, treatment paradigms, survival outcomes, functional outcomes, imaging response, and ongoing challenges. This paper also serves as an introduction to 3 other pLGG manuscripts on (1) pLGG preclinical models, (2) consensus framework for conducting early-phase clinical trials in pLGG, and (3) pLGG resistance, rebound, and recurrence.

Keyword(s): MAPK/ERK pathway ; overview ; pediatric low-grade glioma ; targeted therapy

Note: 2024 Jan 5;26(1):25-37

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Contributing Institute(s):

1. Pädiatische Gliomforschung (B360)
2. KKE Pädiatrische Onkologie (B310)
3. DKTK HD zentral (HD01)

Research Program(s):

1. 312 - Funktionelle und strukturelle Genomforschung (POF4-312) (POF4-312)

Appears in the scientific report 2023

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Database coverage:
; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Essential Science Indicators ; IF >= 15 ; JCR ; Nationallizenz ; PubMed Central ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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