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Molecular genetics and therapeutic targets of pediatric low-grade gliomas

  • Kensuke TateishiEmail author
  • Taishi Nakamura
  • Tetsuya Yamamoto
Original Article

Abstract

Pediatric low-grade gliomas (PLGGs) have relatively favorable prognosis and some resectable PLGGs, such as cerebellar pilocytic astrocytoma, can be cured by surgery alone. However, many PLGG cases are unresectable and some of them undergo tumor progression. Therefore, a multidisciplinary approach is necessary to treat PLGG patients. Recent genomic analysis revealed a broad genomic landscape underlying PLGG. Notably, the majority of PLGGs present MAPK pathway-associated genomic alterations and MAPK signaling-dependent tumor progression. Following preclinical evidence, many clinical trials based on molecular target therapy have been conducted on PLGG patients, some of whom exhibited durable response to target therapy. Here, we provide an overview of PLGG genetics and the evidence supporting the application of molecular target therapy in these patients.

Keywords

Pediatric low-grade glioma Genetic alteration Molecular target therapy 

Abbreviations

PLGG

Pediatric low-grade glioma

PA

Pilocytic astrocytoma

PXA

Pleomorphic xanthoastrocytoma

DNET

Dysembryoplastic neuroepithelial tumor

GG

Ganglioglioma

TSC

Tuberous sclerosis complex

SEGA

Subependymal giant cell astrocytoma

DA

Diffuse astrocytoma

d-OT

Diffuse oligodendroglial tumor

Notes

Compliance with ethical standards

Conflict of interest

The authors declare no potential conflict of interest.

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Copyright information

© The Japan Society of Brain Tumor Pathology 2019

Authors and Affiliations

  • Kensuke Tateishi
    • 1
    • 2
    Email author
  • Taishi Nakamura
    • 1
    • 2
  • Tetsuya Yamamoto
    • 1
  1. 1.Department of NeurosurgeryYokohama City UniversityYokohamaJapan
  2. 2.Neurosurgical-Oncology LabYokohama City UniversityYokohamaJapan

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