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Molecular genetics and therapeutic targets of pediatric low-grade gliomas

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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.

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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

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  1. Kensuke Tateishi and Taishi Nakamura contributed equally.

Authors and Affiliations

  1. Department of Neurosurgery, Yokohama City University, 3-9 Fukuura, Kanazawa, Yokohama, 2360004, Japan

    Kensuke Tateishi, Taishi Nakamura & Tetsuya Yamamoto

  2. Neurosurgical-Oncology Lab, Yokohama City University, Yokohama, 2360004, Japan

    Kensuke Tateishi & Taishi Nakamura

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  1. Kensuke Tateishi
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Tateishi, K., Nakamura, T. & Yamamoto, T. Molecular genetics and therapeutic targets of pediatric low-grade gliomas. Brain Tumor Pathol 36, 74–83 (2019). https://doi.org/10.1007/s10014-019-00340-3

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