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Neuro Oncol. 2017 Feb 1;19(2):153-161. doi: 10.1093/neuonc/now101.

Pediatric high-grade glioma: biologically and clinically in need of new thinking.

Author information

1
Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK.
2
Division of Pediatric Hematology/Oncology, NYU Langone Medical Center, New York, NY, USA.
3
Division of Pediatric Neurooncology, German Cancer Research Centre, Heidelberg, Germany.
4
Pediatric Medical Neuro-Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
5
Department of Neurology & Neurological Sciences, Stanford University, Stanford, California, USA.
6
Department of Developmental Neurobiology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
7
Departments of Pediatrics and Pathology, Preston Robert Tisch Brain Tumor Center, Duke University Medical Center, Durham, North Carolina, USA.
8
Departments of Pediatrics, Neurology, and Neurological Surgery, University of California San Francisco, San Francisco, California, USA.
9
The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, Canada.
10
Neuro-oncology and Experimental Therapeutics, Great Ormond Street Hospital for Children, London, UK.
11
Department of Radiation Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
12
Department of Pediatrics, McGill University, Montreal, Canada.
13
Brain Tumor Program, Texas Children's Cancer Center, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
14
Center for Neuroscience and Behavioral Medicine, Children's National Health System, Washington, District of Columbia, USA.
15
Department of Biochemistry, NYU Langone Medical Center, New York, New York, USA.
16
Key Laboratory of Cell Differentiation and Apoptosis of National Ministry of Education, Department of Pathophysiology, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
17
Department of Pediatric Hematology and Oncology, Heidelberg University Hospital, Heidelberg, Germany.

Abstract

High-grade gliomas in children are different from those that arise in adults. Recent collaborative molecular analyses of these rare cancers have revealed previously unappreciated connections among chromatin regulation, developmental signaling, and tumorigenesis. As we begin to unravel the unique developmental origins and distinct biological drivers of this heterogeneous group of tumors, clinical trials need to keep pace. It is important to avoid therapeutic strategies developed purely using data obtained from studies on adult glioblastoma. This approach has resulted in repetitive trials and ineffective treatments being applied to these children, with limited improvement in clinical outcome. The authors of this perspective, comprising biology and clinical expertise in the disease, recently convened to discuss the most effective ways to translate the emerging molecular insights into patient benefit. This article reviews our current understanding of pediatric high-grade glioma and suggests approaches for innovative clinical management.

KEYWORDS:

DIPG; clinical trials; genomics; glioma; pediatric

PMID:
27282398
PMCID:
PMC5464243
DOI:
10.1093/neuonc/now101
[Indexed for MEDLINE]
Free PMC Article

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