Pediatric low-grade gliomas: implications of the biologic era.
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.
Center for Neuroscience and Behavioral Medicine, Washington, District of Columbia, USA.|Gilbert Family Neurofibromatosis Institute, Washington, District of Columbia, USA.|Brain Tumor Institute, Washington, District of Columbia, USA.
Roger J
RJ
Packer
Division of Pediatric Neuro-oncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.|Department of Pediatric Hematology and Oncology, Heidelberg University Hospital, Heidelberg, Germany.
Stephan
S
Pfister
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.
Eric
E
Bouffet
Center for Neuroscience and Behavioral Medicine, Washington, District of Columbia, USA.|Gilbert Family Neurofibromatosis Institute, Washington, District of Columbia, USA.|Brain Tumor Institute, Washington, District of Columbia, USA.
Robert
R
Avery
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.
Pratiti
P
Bandopadhayay
Center for Neuroscience and Behavioral Medicine, Washington, District of Columbia, USA.|Gilbert Family Neurofibromatosis Institute, Washington, District of Columbia, USA.|Brain Tumor Institute, Washington, District of Columbia, USA.|Center for Cancer and Immunology Research, Washington, District of Columbia, USA.
Miriam
M
Bornhorst
Department of Pediatrics, UT Southwestern Medical School, Dallas, Texas, USA.
Daniel C
DC
Bowers
Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee. USA.|Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
David
D
Ellison
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.
Jason
J
Fangusaro
Northwestern Feinberg School of Medicine, Chicago, Illinois; Children's Hospital Colorado, University of Colorado, Aurora, Colorado, USA.
Nicholas
N
Foreman
Brain Tumor Center, Brain Tumor Translational Research, UC Department of Pediatrics, Cincinnati, Ohio, USA.
Maryam
M
Fouladi
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
Amar
A
Gajjar
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.
Daphne
D
Haas-Kogan
Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee. USA.
Cynthia
C
Hawkins
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
Cheng-Ying
CY
Ho
Center for Neuroscience and Behavioral Medicine, Washington, District of Columbia, USA.|Brain Tumor Institute, Washington, District of Columbia, USA.|Center for Cancer and Immunology Research, Washington, District of Columbia, USA.
Eugene
E
Hwang
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.
Nada
N
Jabado
Brain Tumor Institute, Washington, District of Columbia, USA.|Center for Cancer and Immunology Research, Washington, District of Columbia, USA.
Lindsay B
LB
Kilburn
Northwestern Feinberg School of Medicine, Chicago, Illinois; Children's Hospital Colorado, University of Colorado, Aurora, Colorado, USA.
Alvaro
A
Lassaletta
Brain Tumor Center, Brain Tumor Translational Research, UC Department of Pediatrics, Cincinnati, Ohio, USA.|Brain Tumor Center, Brain Tumor Translational Research, UC Department of Pediatrics, Cincinnati, Ohio, USA.
Keith L
KL
Ligon
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.|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.
Maura
M
Massimino
Emma Children's Hospital AMC, Amsterdam-Zuidoost, Netherlands.
Schouten-van
SV
Meeteren
Department of Neurology, Pediatrics and Neurosurgery, University of California San Francisco, San Francisco, California, USA.
Sabine
S
Mueller
Department of Neurology, Pediatrics and Neurosurgery, University of California San Francisco, San Francisco, California, USA.
Theo
T
Nicolaides
Department of Woman's and Child's Health, University of Padua, Padua, Italy.
Giorgio
G
Perilongo
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.
Uri
U
Tabori
Gilbert Family Neurofibromatosis Institute, Washington, District of Columbia, USA.|Brain Tumor Institute, Washington, District of Columbia, USA.|Division of Neuroradiology, Washington, District of Columbia, USA.
Gilbert
G
Vezina
National Cancer Institute, Pediatric Oncology and Neuro-Oncology Branches, Bethesda, Maryland, USA.
Katherine
K
Warren
Division of Pediatric Neuro-oncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
Olaf
O
Witt
Center for Neuroscience and Behavioral Medicine, Washington, District of Columbia, USA.|Gilbert Family Neurofibromatosis Institute, Washington, District of Columbia, USA.|Center for Cancer and Immunology Research, Washington, District of Columbia, USA.
Yuan
Y
Zhu
Division of Pediatric Neuro-oncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
David T
DT
Jones
Brain Tumor Center, Brain Tumor Translational Research, UC Department of Pediatrics, Cincinnati, Ohio, USA.
Mark
M
Kieran
D001932:Brain Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D002648:Child; D005910:Glioma / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D006801:Humans; D058990:Molecular Targeted Therapy; D015398:Signal Transduction
low-grade glioma; neurofibromatosis type 1; pediatric brain tumor; pilocytic astrocytoma; RAS/MAPK pathway
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