Format

Send to

Choose Destination
Neuro Oncol. 2018 Jan 22;20(2):160-173. doi: 10.1093/neuonc/nox141.

Pediatric low-grade gliomas: next biologically driven steps.

Author information

1
Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Hopp Children's Cancer Center, Heidelberg, Germany.
2
Department of Medical Oncology, Brigham and Women's Hospital, Harvard Medical School, and the Broad Institutem, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts, USA.
3
Paediatric Neuro-Oncology Program, Research Institute, The Arthur and Sonia Labatt Brain Tumour Research Centre, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
4
Department of Pathology, Harvard Medical School, Boston Children's Hospital, Boston, Massachusetts, USA.
5
Gilbert Family Neurofibromatosis Institute, Brain Tumor Institute, Children's National Health System, Washington DC, USA.
6
Center for Cancer and Immunology Research, Children's National Health System, Washington DC, USA.
7
Department of Pathology and Department of Oncology, St Jude Children's Research Hospital, Memphis, Tennessee, USA.
8
Ann and Robert H. Lurie Children's Hospital of Chicago Department of Pediatric Hematology/Oncology and Stem Cell Transplantation, Northwestern Feinberg School of Medicine, Chicago, Illinois, USA.
9
Department of Pediatric Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
10
Children's Hospital Colorado, University of Colorado, Aurora, Colorado, USA.
11
Brain Tumor Center, Brain Tumor Translational Research and Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio, USA.
12
Neuro-oncology and Experimental Therapeutics, Great Ormond Street Hospital for Children, London, UK.
13
Division of Pathology, The Arthur and Sonia Labatt Brain Tumor Research Centre, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
14
Department of Pediatrics, McGill University, Montreal, Quebec, Canada.
15
Pediatric Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
16
Department of Neurology, Pediatrics, and Neurosurgery, University of California San Francisco, San Francisco, California, USA.
17
Department of Woman's and Child's Health, University of Padua, Padua, Italy.
18
Emma Children's Hospital AMC, Amsterdam-Zuidoost, Netherlands.
19
National Cancer Institute, Pediatric Oncology and Neuro-Oncology Branches, Bethesda, Maryland, USA.
20
Children's Brain Tumor Research Centre, QMC University of Nottingham, Nottingham, UK.
21
Department of Pediatrics, UT Southwestern Medical School, Dallas, Texas, USA.
22
Center for Neuroscience and Behavioral Medicine, Gilbert Family Neurofibromatosis Institute, Brain Tumor Institute, Children's National Health System, Washington DC, USA.

Abstract

Despite the fact that they are not typically life-threatening, low-grade gliomas (LGGs) remain a significant clinical challenge in pediatric neuro-oncology due to comorbidities associated with these tumors and/or their treatments, and their propensity to multiply recurs. LGGs, in total the most common brain tumors arising in childhood, can often become a chronic problem requiring decades of management. The Second International Consensus Conference on Pediatric Low-Grade Gliomas held in Padua, Italy in 2016 was convened in an attempt to advance the pace of translating biological discoveries on LGGs into meaningful clinical benefit. Topics discussed included: the implications of our growing biological understanding of the genomics underlying these tumors; the assessment of the model systems available; the implications of the molecular and histopathologic differences between adult and pediatric diffuse gliomas; and steps needed to expedite targeted therapy into late-stage clinical trials for newly diagnosed cases. Methods for the diagnostic assessment of alterations in the Ras/mitogen-activated protein kinase pathway, typical for these tumors, were also considered. While the overall tone was positive, with a consensus that progress is being and will continue to be made, the scale of the challenge presented by this complex group of tumors was also acknowledged. The conclusions and recommendations of the meeting panel are provided here as an outline of current thinking and a basis for further discussion.

KEYWORDS:

MAPK pathway; low-grade glioma; molecular diagnostics; neurooncology; pediatric brain tumor; targeted therapy

PMID:
29016845
PMCID:
PMC5786244
DOI:
10.1093/neuonc/nox141
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Silverchair Information Systems Icon for PubMed Central
Loading ...
Support Center