Chordoid glioma
ORPHA:251674· ICD-10 C71.9
Definition
A rare central nervous system tumor characterized by a non-infiltrative and well-circumscribed WHO grade 2 glial neoplasm occurring in the third ventricle, often extending to hypothalamus. Chordoid glioma presents most frequently in middle-aged women with symptoms of memory deficits, headaches and visual loss, related to its location. Virtually all chordoid gliomas exhibit positivity for GFAP and TTF1 immunostaining, PRKCAp.D463H mutation, and a specific chordoid glioma DNA methylation class.
- Prevalence
- <1 / 1 000 000
- Age of onset
- Adult