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Neuro Oncol. 2017 Jun 1;19(6):750-761. doi: 10.1093/neuonc/now209.

Pediatric low-grade gliomas: implications of the biologic era.

Author information

1
Center for Neuroscience and Behavioral Medicine, Washington, District of Columbia, USA.
2
Gilbert Family Neurofibromatosis Institute, Washington, District of Columbia, USA.
3
Brain Tumor Institute, Washington, District of Columbia, USA.
4
Division of Pediatric Neuro-oncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
5
Department of Pediatric Hematology and Oncology, Heidelberg University Hospital, Heidelberg, Germany.
6
Paediatric Neuro-Oncology Program, Research Institute and The Arthur and Sonia Labatt Brain Tumour Research Centre, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
7
Department of Pediatrics, Brigham and Women's Hospital, Harvard Medical School, and the Broad Institute, Dana-Farber/Boston Children's Cancer and Blood Disorders Centre, Boston, Massachusetts, USA.
8
Center for Cancer and Immunology Research, Washington, District of Columbia, USA.
9
Department of Pediatrics, UT Southwestern Medical School, Dallas, Texas, USA.
10
Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee. USA.
11
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
12
Ann and Robert H. Lurie Children's Hospital of Chicago Department of Pediatric Hematology/Oncology and Stem Cell Transplantation, University of Colorado, Aurora, Colorado, USA.
13
Northwestern Feinberg School of Medicine, Chicago, Illinois; Children's Hospital Colorado, University of Colorado, Aurora, Colorado, USA.
14
Brain Tumor Center, Brain Tumor Translational Research, UC Department of Pediatrics, Cincinnati, Ohio, USA.
15
Department of Radiation Oncology, Brigham and Women's Hospital, Harvard Medical School, and the Broad Institute, Dana-Farber/Boston Children's Cancer and Blood Disorders Centre, Boston, Massachusetts, USA.
16
Emma Children's Hospital AMC, Amsterdam-Zuidoost, Netherlands.
17
Department of Neurology, Pediatrics and Neurosurgery, University of California San Francisco, San Francisco, California, USA.
18
Department of Woman's and Child's Health, University of Padua, Padua, Italy.
19
Division of Haematology/Oncology, Research Institute and The Arthur and Sonia Labatt Brain Tumour Research Centre, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
20
Division of Neuroradiology, Washington, District of Columbia, USA.
21
National Cancer Institute, Pediatric Oncology and Neuro-Oncology Branches, Bethesda, Maryland, USA.

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 classifications and, more importantly, for future treatment of pediatric LGGs are summarized here. There is uniform agreement that molecular characterization must be incorporated into classification and is increasingly critical for appropriate management. Molecular-targeted 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.

KEYWORDS:

low-grade glioma; neurofibromatosis type 1; pediatric brain tumor; pilocytic astrocytoma; RAS/MAPK pathway

PMID:
27683733
PMCID:
PMC5464436
DOI:
10.1093/neuonc/now209
[Indexed for MEDLINE]
Free PMC Article

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