AUTHOR=Ceglie Giulia , Del Baldo Giada , Agolini Emanuele , Rinelli Martina , Cacchione Antonella , Del Bufalo Francesca , Vinci Maria , Carta Roberto , Boccuto Luigi , Miele Evelina , Mastronuzzi Angela , Locatelli Franco , Carai Andrea 

TITLE=Cancer Predisposition Syndromes Associated With Pediatric High-Grade Gliomas

JOURNAL=Frontiers in Pediatrics

VOLUME=8

YEAR=2020

URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2020.561487

DOI=10.3389/fped.2020.561487

ISSN=2296-2360

ABSTRACT=<p>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 guide for suspecting these syndromes and referring for genetic counseling.</p>