An Update on Neurofibromatosis Type 1-Associated Gliomas

Mina Lobbous; Joshua D Bernstock; Elizabeth Coffee; Gregory K Friedman; Laura K Metrock; Gustavo Chagoya; Galal Elsayed; Ichiro Nakano; James R Hackney; Bruce R Korf; Louis B Nabors

doi:10.3390/cancers12010114

An Update on Neurofibromatosis Type 1-Associated Gliomas

Cancers (Basel). 2020 Jan 1;12(1):114. doi: 10.3390/cancers12010114.

Authors

Affiliations

¹ Division of Neuro Oncology, Department of Neurology, University of Alabama at Birmingham, 510 20th Street South, Faculty Office Tower suite 1020 Birmingham, Birmingham, AL 35294, USA.
² Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA.
³ Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
⁴ Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
⁵ Department of Pathology, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
⁶ Department of Genetics, University of Alabama at Birmingham, Birmingham, AL 35294, USA.

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor predisposition syndrome that affects children and adults. Individuals with NF1 are at high risk for central nervous system neoplasms including gliomas. The purpose of this review is to discuss the spectrum of intracranial gliomas arising in individuals with NF1 with a focus on recent preclinical and clinical data. In this review, possible mechanisms of gliomagenesis are discussed, including the contribution of different signaling pathways and tumor microenvironment. Furthermore, we discuss the recent notable advances in the developing therapeutic landscape for NF1-associated gliomas including clinical trials and collaborative efforts.

Keywords: astrocytoma; glioblastoma; gliomas; neurofibromatosis; therapeutics.

Publication types

Review

Grants and funding

R01 NS107071/NS/NINDS NIH HHS/United States