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

Author:

Packer Roger J12,Iavarone Antonio3,Jones David T W4,Blakeley Jaishri O5,Bouffet Eric6,Fisher Michael J7,Hwang Eugene2,Hawkins Cynthia6,Kilburn Lindsay2,MacDonald Tobey8,Pfister Stefan M4,Rood Brian2,Rodriguez Fausto J9,Tabori Uri6,Ramaswamy Vijay6ORCID,Zhu Yuan2,Fangusaro Jason8,Johnston Stephen A10,Gutmann David H11

Affiliation:

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

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.

Funder

Gilbert Family Foundation

Publisher

Oxford University Press (OUP)

Subject

Cancer Research,Neurology (clinical),Oncology

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