Cancer Predisposition Syndromes Associated With Pediatric High-Grade Gliomas.
Ontology highlight
ABSTRACT:
SUBMITTER: Ceglie G
PROVIDER: S-EPMC7690624 | biostudies-literature | 2020
REPOSITORIES: biostudies-literature
Publications
Cancer Predisposition Syndromes Associated With Pediatric High-Grade Gliomas.
Ceglie Giulia G Del Baldo Giada G Agolini Emanuele E Rinelli Martina M Cacchione Antonella A Del Bufalo Francesca F Vinci Maria M Carta Roberto R Boccuto Luigi L Miele Evelina E Mastronuzzi Angela A Locatelli Franco F Carai Andrea A
Frontiers in pediatrics 20201112
Pediatric High-Grade Gliomas (pHGG) are among the deadliest childhood brain tumors and can be associated with an underlying cancer predisposing syndrome. The thorough understanding of these syndromes can aid the clinician in their prompt recognition, leading to an informed genetic counseling for families and to a wider understanding of a specific genetic landscape of the tumor for target therapies. In this review, we summarize the main pHGG-associated cancer predisposing conditions, providing a ...[more]
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Project description:Pediatric high grade gliomas (HGG) are lethal tumors which are currently untreatable. A number of recent studies have provided much needed insights into the mutations and mechanisms which drive oncogenesis in pediatric HGGs. It is now clear that mutations in chromatin proteins, particularly H3.3 and its associated chaperone complex (ATRX), are a hallmark feature of pediatric HGGs. We review the current literature on the normal roles of the ATRX/H3.3 complex and how these functions are disrupted by oncogenic mutations. We discuss the current clinical trials and pre-clinical models that target chromatin and DNA, and how these agents fit into the ATRX/H3.3 mutation model. As chromatin mutations are a relatively new discovery in pediatric HGGs, developing clear mechanistic insights are a key step to improving therapies for these tumors.
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