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A rare, slow-growing neuroepithelial neoplasm of uncertain origin affecting adults. It is located in the third ventricle. It is characterized by the presence of epithelioid cells which express GFAP, and mucinous stroma which contains lymphoplasmacytic infiltrates.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
1 clinical trial registered. Interventions under study include drug therapy. Pipeline includes 1 PHASE4. Research is primarily industry-sponsored.
8 publications have been identified in PubMed for chordoid glioma of the third ventricle. Research spans Case Report / Case Series (63%), Basic Science / Preclinical (25%), and Review / Meta-Analysis (13%).
Pham D (2026). [PMID: 42130597](https://pubmed.ncbi.nlm.nih.gov/42130597/). *Int J Surg Case Rep*. [Case Report / Case Series]
Nayak KK (2025). [PMID: 40212193](https://pubmed.ncbi.nlm.nih.gov/40212193/). *Annals of medicine and surgery (2012)*. [Case Report / Case Series]
Cuccia F (2025). [PMID: 41333225](https://pubmed.ncbi.nlm.nih.gov/41333225/). *Frontiers in oncology*. [Basic Science / Preclinical]
Bellamy C (2025). [PMID: 40501730](https://pubmed.ncbi.nlm.nih.gov/40501730/). *bioRxiv : the preprint server for biology*. [Case Report / Case Series]
Shoji K (2025). [PMID: 41181189](https://pubmed.ncbi.nlm.nih.gov/41181189/). *NMC case report journal*. [Case Report / Case Series]
Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 5:38 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Feng L (2025). [PMID: 39970814](https://pubmed.ncbi.nlm.nih.gov/39970814/). *Pathology, research and practice*. [Case Report / Case Series]
Calleja V (2025). [PMID: 41187221](https://pubmed.ncbi.nlm.nih.gov/41187221/). *Science signaling*. [Basic Science / Preclinical]
Esteban-Rodríguez I (2024). [PMID: 37668299](https://pubmed.ncbi.nlm.nih.gov/37668299/). *Cytopathology : official journal of the British Society for Clinical Cytology*. [Review / Meta-Analysis]