Pediatric low-grade gliomas: Implications of the biologic era


Roger J. Packer, Childrens National Health System
Stephan Pfster, Gilbert Family Neurofbromatosis Institute
Eric Bouffet, Center for Cancer and Immunology Research
Robert Avery, Division of Pathology
Pratiti Bandopadhayay, Brain Tumor Institute
Miriam Bornhorst, Division of Neuroradiology
Daniel C. Bowers, Childrens National Health System
David Ellison, Brain Tumor Institute
Jason Fangusaro, Childrens National Health System
Nicholas Foreman, German Cancer Research Center
Maryam Fouladi, Childrens National Health System
Amar Gajjar, Childrens National Health System
Daphne Haas-Kogan, Paediatric Neuro-Oncology Program
Cynthia Hawkins, Division of Pathology
Cheng Ying Ho, Brain Tumor Institute
Eugene Hwang, Center for Cancer and Immunology Research
Nada Jabado, Childrens National Health System
Lindsay B. Kilburn, Center for Cancer and Immunology Research
Alvaro Lassaletta, Division of Neuroradiology
Keith L. Ligon
Maura Massimino, Center for Cancer and Immunology Research
Schouten Van Meeteren, Brigham and Women's Hospital
Sabine Mueller, UT Southwestern Medical School
Theo Nicolaides, Childrens National Health System
Giorgio Perilongo
Uri Tabori, St. Jude Children's Research Hospital
Gilbert Vezina, Ann and Robert H. Lurie Children's Hospital of Chicago
Katherine Warren, Northwestern University Feinberg School of Medicine
Olaf Witt, Brain Tumor Institute
Yuan Zhu, Center for Cancer and Immunology Research
David T. Jones, Childrens National Health System
Mark Kieran, Childrens National Health System

Document Type

Journal Article

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Low-grade glioma; Neurofbromatosis type 1; Pediatric brain tumor; Pilocytic astrocytoma; RAS/MAPK pathway


For the past decade, it has been recognized that pediatric low-grade gliomas (LGGs) and glial-neuronal tumors carry distinct molecular alterations with resultant aberrant intracellular signaling in the Ras-mitogen-activated protein kinase pathway. The conclusions and recommendations of a consensus conference of how best to integrate the growing body of molecular genetic information into tumor classifcations and, more importantly, for future treatment of pediatric LGGs are summarized here. There is uniform agreement that molecular characterization must be incorporated into classifcation and is increasingly critical for appropriate management. Moleculartargeted therapies should be integrated expeditiously, but also carefully into the management of these tumors and success measured not only by radiographic responses or stability, but also by functional outcomes. These trials need to be carried out with the caveat that the long-term impact of molecularly targeted therapy on the developing nervous system, especially with long duration treatment, is essentially unknown.