Title

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

Document Type

Journal Article

Publication Date

6-9-2020

Journal

Neuro-Oncology

Volume

22

Issue

6

DOI

10.1093/neuonc/noaa036

Keywords

gliomas; immunotherapy; molecular-targeted therapy; neurofibromatosis type 1; pilocytic astrocytomas

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.

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