Pediatric low-grade gliomas: Implications of the biologic era

Roger J. Packer, Stephan Pfster, Eric Bouffet, Robert Avery, Pratiti Bandopadhayay, Miriam Bornhorst, Daniel C. Bowers, David Ellison, Jason Fangusaro, Nicholas Foreman, Maryam Fouladi, Amar Gajjar, Daphne Haas-Kogan, Cynthia Hawkins, Cheng Ying Ho, Eugene Hwang, Nada Jabado, Lindsay B. Kilburn, Alvaro Lassaletta, Keith L. LigonMaura Massimino, Schouten Van Meeteren, Sabine Mueller, Theo Nicolaides, Giorgio Perilongo, Uri Tabori, Gilbert Vezina, Katherine Warren, Olaf Witt, Yuan Zhu, David T. Jones, Mark Kieran

Research output: Contribution to journalArticle

39 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)750-761
Number of pages12
JournalNeuro-Oncology
Volume19
Issue number6
DOIs
StatePublished - 2017

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Keywords

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

ASJC Scopus subject areas

  • Oncology
  • Clinical Neurology
  • Cancer Research

Cite this

Packer, R. J., Pfster, S., Bouffet, E., Avery, R., Bandopadhayay, P., Bornhorst, M., Bowers, D. C., Ellison, D., Fangusaro, J., Foreman, N., Fouladi, M., Gajjar, A., Haas-Kogan, D., Hawkins, C., Ho, C. Y., Hwang, E., Jabado, N., Kilburn, L. B., Lassaletta, A., ... Kieran, M. (2017). Pediatric low-grade gliomas: Implications of the biologic era. Neuro-Oncology, 19(6), 750-761. https://doi.org/10.1093/neuonc/now209