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

doi:10.3389/fped.2020.561487

Cancer Predisposition Syndromes Associated With Pediatric High-Grade Gliomas

Front Pediatr. 2020 Nov 12:8:561487. doi: 10.3389/fped.2020.561487. eCollection 2020.

Authors

Affiliations

¹ Department of Pediatric Hematology/Oncology and Cell and Gene Therapy, Istituto di Ricovero e Cura a Carattere Scientifico Bambino Gesù Children's Hospital, Rome, Italy.
² Laboratory of Medical Genetics, Istituto di Ricovero e Cura a Carattere Scientifico Bambino Gesù Children's Hospital, Rome, Italy.
³ Greenwood Genetic Center, Greenwood, SC, United States.
⁴ Clemson University School of Health Research, Clemson, SC, United States.
⁵ Sapienza, University of Rome, Rome, Italy.
⁶ Neurosurgery Unit, Department of Neurological and Psychiatric Sciences, Istituto di Ricovero e Cura a Carattere Scientifico Bambino Gesù Children's Hospital, Rome, Italy.

Abstract

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.

Keywords: brain tumors; cancer predisposition; genetics of cancer; high grade gliomas; pediatric neuro- oncology.

Publication types

Review