Cancer Predisposition Syndromes Associated With Pediatric High-Grade Gliomas

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

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish
Article number561487
JournalFrontiers in Pediatrics
Publication statusPublished - Nov 12 2020


  • brain tumors
  • cancer predisposition
  • genetics of cancer
  • high grade gliomas
  • pediatric neuro- oncology

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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