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

Roger J Packer; Antonio Iavarone; David T W Jones; Jaishri O Blakeley; Eric Bouffet; Michael J Fisher; Eugene Hwang; Cynthia Hawkins; Lindsay Kilburn; Tobey MacDonald; Stefan M Pfister; Brian Rood; Fausto J Rodriguez; Uri Tabori; Vijay Ramaswamy; Yuan Zhu; Jason Fangusaro; Stephen A Johnston; David H Gutmann

doi:10.1093/neuonc/noaa036

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

Neuro Oncol. 2020 Jun 9;22(6):773-784. doi: 10.1093/neuonc/noaa036.

Authors

Roger J Packer^{1

2}, Antonio Iavarone³, David T W Jones⁴, Jaishri O Blakeley⁵, Eric Bouffet⁶, Michael J Fisher⁷, Eugene Hwang², Cynthia Hawkins⁶, Lindsay Kilburn², Tobey MacDonald⁸, Stefan M Pfister⁴, Brian Rood², Fausto J Rodriguez⁹, Uri Tabori⁶, Vijay Ramaswamy⁶, Yuan Zhu², Jason Fangusaro⁸, Stephen A Johnston¹⁰, David H Gutmann¹¹

Affiliations

¹ Center for Neuroscience and Behavioral Medicine, Washington, DC, USA.
² Gilbert Family Neurofibromatosis Institute, Brain Tumor Institute, and Children's National Hospital, Washington, DC, USA.
³ Departments of Neurology and Pathology Institute for Cancer Genetics Columbia University Medical Center, New York, New York, USA.
⁴ Division of Pediatric Neuro-Oncology German Cancer Research Center Hopp Children's Cancer Center Heidelberg, Germany.
⁵ Departments of Neurology; Oncology; Neurosurgery, Baltimore, Maryland, USA.
⁶ Pediatric Neuro-Oncology Program; Research Institute; and The Arthur and Sonia Labatt; Brain Tumor Research Centre, Hospital for Sick Children, Toronto, Canada.
⁷ Department of Pediatric Oncology; Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
⁸ Department of Pediatrics; Emory University School of Medicine, Atlanta, Georgia, USA.
⁹ Pathology; The Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
¹⁰ Center for Innovations in Medicine; Biodesign Institute; Arizona State University, Tempe, Arizona, USA.
¹¹ Department of Neurology; Washington University, St Louis, Missouri, USA.

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.

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

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Animals
Brain Neoplasms* / diagnosis
Brain Neoplasms* / therapy
Child
Disease Models, Animal
Glioma* / diagnosis
Glioma* / therapy
Humans
Neurofibromatosis 1* / diagnosis
Neurofibromatosis 1* / therapy