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Updated: Feb 14, 2026

Primary Orthotopic Glioma Xenografts Recapitulate Infiltrative Growth and Isocitrate Dehydrogenase I Mutation
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A recurrent kinase domain mutation in PRKCA defines chordoid glioma of the third ventricle

Benjamin Goode1, Gourish Mondal1, Michael Hyun1

  • 1Department of Pathology, University of California, San Francisco, CA, 94143, USA.

Nature Communications
|February 25, 2018
PubMed

Insights

Chordoid glioma, a rare brain tumor, harbors a specific PRKCA mutation. This discovery identifies a potential therapeutic target for this challenging cancer.

Area of Science:

  • Neuro-oncology
  • Cancer Genomics

Background:

  • Chordoid glioma is a rare brain tumor originating from the lamina terminalis.
  • Despite low-grade histology, these tumors have poor outcomes due to location and lack of effective therapies.

Purpose of the Study:

  • To investigate the genomic landscape of chordoid glioma.
  • To identify potential therapeutic targets for chordoid glioma.

Main Methods:

  • Genomic profiling of 13 chordoid glioma samples.
  • In vitro functional studies using immortalized human astrocytes expressing mutant PRKCA.

Main Results:

  • A recurrent D463H missense mutation in PRKCA was identified in all chordoid gliomas.
  • Mutant PRKCA expression increased phospho-ERK and anchorage-independent growth.
  • MEK inhibition blocked the effects of mutant PRKCA.

Conclusions:

  • PRKCA is a recurrently mutated oncogene in chordoid glioma.
  • Targeting MEK presents a potential therapeutic strategy for chordoid glioma.

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