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Pediatric low-grade gliomas: next biologically driven steps.

David T W Jones1, Mark W Kieran2, Eric Bouffet3

  • 1Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Hopp Children's Cancer Center, Heidelberg, Germany.

Neuro-Oncology
|October 11, 2017
PubMed
Summary

Pediatric low-grade gliomas (LGGs) are the most common childhood brain tumors, posing chronic management challenges. A consensus conference highlighted progress in understanding LGG genomics and advancing targeted therapies.

Keywords:
MAPK pathwaylow-grade gliomamolecular diagnosticsneurooncologypediatric brain tumortargeted therapy

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Area of Science:

  • Pediatric Neuro-Oncology
  • Cancer Genomics
  • Translational Medicine

Background:

  • Low-grade gliomas (LGGs) represent the most common pediatric brain tumors.
  • Despite not being typically life-threatening, LGGs present significant clinical challenges due to comorbidities, treatment side effects, and recurrence.
  • LGGs often require lifelong management, underscoring the need for improved therapeutic strategies.

Purpose of the Study:

  • To advance the translation of biological discoveries into clinical benefits for pediatric low-grade gliomas.
  • To discuss the implications of genomic understanding, model systems, and molecular differences between pediatric and adult gliomas.
  • To outline steps for expediting targeted therapies into clinical trials for newly diagnosed pediatric LGGs.

Main Methods:

  • Convened the Second International Consensus Conference on Pediatric Low-Grade Gliomas in 2016.
  • Discussed the biological understanding, genomics, and model systems for pediatric LGGs.
  • Considered diagnostic methods for Ras/mitogen-activated protein kinase pathway alterations.

Main Results:

  • Acknowledged progress in understanding pediatric LGG biology and genomics.
  • Identified the need to expedite targeted therapies into late-stage clinical trials.
  • Highlighted the complexity and chronic nature of managing pediatric LGGs.

Conclusions:

  • Progress is being made in pediatric low-grade glioma research and treatment.
  • Further discussion and action are needed to address the challenges posed by these tumors.
  • The conference provided a basis for future research and clinical management strategies.