Implications of new understandings of gliomas in children and adults with NF1: report of a consensus conference

• Type of Material: Article
• Authors: Roger J. Packer, Children's National Hospital; Antonio Iavarone, Columbia University; David T. W. Jones, German Cancer Research Center; Joishri O. Blakeley, Johns Hopkins University; Eric Bouffet, Hospital for Sick Children; Michael J. Fisher, Childrens Hospital of Philadelphia; Eugene Hwang, Children's National Hospital; Cynthia Hawkins, Hospital for Sick Children; Lindsay Kilburn, Children's National Hospital; Tobey MacDonald, Emory University; Stefan M. Pfister, German Cancer Research Center; Brian Rood, Children's National Hospital; Fausto J. Rodriguez, Johns Hopkins University; Uri Tabori, Hospital for Sick Children; Vijay Ramaswamy, Hospital for Sick Children; Yuan Zhu, Children's National Hospital; Jason Fangusaro, Emory University; Stephen A. Johnston, Arizona State University Tempe; David H. Gutmann, Washington University in St. Louis
• Language: English
• Date: 2020-06-01
• Publisher: Oxford University Press (OUP): Policy B - Oxford Open Option F
• Publication Version: Final Published Version
• Copyright Statement: © 2020 The Author(s) 2020. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.
• License: Creative Commons Attribution-NonCommercial 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1093/neuonc/noaa036
• Title of Journal or Parent Work: Neuro-Oncology
• ISSN: 1522-8517
• Volume: 22
• Issue: 6
• Start Page: 773
• End Page: 784
• Grant/Funding Information: Financial support has been provided by the Gilbert Family Foundation (EIN number 81-0810541; Bridge number 0912232251).
• Keywords: PEDIATRIC-PATIENTS; TUMOR; pilocytic astrocytomas; Life Sciences & Biomedicine; molecular-targeted therapy; REVEALS; neurofibromatosis type 1; RECURRENT; NEUROFIBROMATOSIS TYPE-1; immunotherapy; Clinical Neurology; MICROGLIA; Science & Technology; OPTIC PATHWAY GLIOMAS; Neurosciences & Neurology; PHASE-II; LOW-GRADE GLIOMAS; EXPRESSION; gliomas; Oncology
• Research Categories: Health Sciences, Oncology

Abstract

Gliomas are the most common primary central nervous system tumors occurring in children and adults with neurofibromatosis type 1 (NF1). Over the past decade, discoveries of the molecular basis of low-grade gliomas (LGGs) have led to new approaches for diagnosis and treatments. However, these new understandings have not been fully applied to the management of NF1-associated gliomas. A consensus panel consisting of experts in NF1 and gliomas was convened to review the current molecular knowledge of NF1-associated low-grade "transformed" and high-grade gliomas; insights gained from mouse models of NF1-LGGs; challenges in diagnosing and treating older patients with NF1-associated gliomas; and advances in molecularly targeted treatment and potential immunologic treatment of these tumors. Next steps are recommended to advance the management and outcomes for NF1-associated gliomas.