Ependymal, choroid plexus and glioneuronal tumours
- Lesion
Tumours from the ventricle lining and choroid plexus block CSF flow; mixed neuronal tumours are usually slow and cause seizures.
What it is
Ependymomas arise from cells lining the ventricles and central canal: in children usually in the fourth ventricle, in adults often in the spinal cord. They are now classified by site and molecular group. Choroid plexus papillomas, mostly in the lateral ventricles of children, can overproduce CSF; their malignant counterpart, choroid plexus carcinoma, is linked to TP53 (Li-Fraumeni). Neuronal and mixed glioneuronal tumours, such as ganglioglioma and dysembryoplastic neuroepithelial tumour, are low-grade and a common cause of drug-resistant epilepsy.
Ventricular and glioneuronal tumours
| Tumour | Usual site | Histology clue | Typical presentation |
|---|---|---|---|
| Ependymoma | Fourth ventricle (children), spinal cord (adults) | Perivascular pseudorosettes, true rosettes | Hydrocephalus or cord symptoms |
| Myxopapillary ependymoma | Filum terminale | Papillae with myxoid cores | Back pain |
| Subependymoma | Fourth or lateral ventricle | Clustered nuclei in dense glial matrix | Often silent |
| Choroid plexus papilloma | Lateral ventricle (children), fourth ventricle (adults) | Fronds of plexus-like epithelium | Hydrocephalus |
| Choroid plexus carcinoma | Lateral ventricle in young children | Solid sheets, atypia, invasion | Li-Fraumeni association |
| Ganglioglioma | Temporal lobe | Dysmorphic neurons with glial cells | Epilepsy |
| Dysembryoplastic neuroepithelial tumour | Temporal cortex | Floating neurons in mucoid matrix | Epilepsy |
| Central neurocytoma | Lateral ventricle near foramen of Monro | Uniform round neuronal cells, synaptophysin positive | Hydrocephalus in young adults |
Markers
- GFAP
- EMA dot-like staining (ependymoma)
- Synaptophysin, NeuN (neuronal tumours)
- CD34 (ganglioglioma)
- BRAF V600E
Clinical notes
- Fourth ventricle tumours cause hydrocephalus with morning headache, vomiting and ataxia.
- Removing a glioneuronal tumour often cures the associated epilepsy.
Test yourself
Think of the answer, then tap the question to see it.
What is a perivascular pseudorosette?
Tumour cells arranged around a blood vessel with a cell-free zone of fibrillary processes between, typical of ependymoma.
Where does an ependymoma usually arise in a child?
In the fourth ventricle, in the posterior fossa.
Which tumours commonly cause long-standing drug-resistant epilepsy in young people?
Low-grade glioneuronal tumours such as ganglioglioma and dysembryoplastic neuroepithelial tumour.
Elsewhere in DurgiPedia
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Childhood Ependymoma Treatment (PDQ®)–Health Professional Version (National Cancer Institute, 2026); Childhood Astrocytomas, Other Gliomas, and Glioneuronal/Neuronal Tumors Treatment (PDQ®)–Health Professional Version (National Cancer Institute, 2026).
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Representative model, for educational purposes only.