Related Subjects:
|Brain tumour s
|Astrocytomas
|Brain Metastases
|Tuberous sclerosis
|Turcot's syndrome
|Lhermitte Duclos Disease
|Oligodendroglioma
|Acute Hydrocephalus
|Intracranial Hypertension
|Primary CNS Lymphoma (PCNSL)
🧠 About
- Ependymoma is a central nervous system (CNS) tumour arising from the ependymal cells lining the ventricles and the central canal of the spinal cord.
- It is considered one of the more treatable gliomas due to its relatively circumscribed nature compared to infiltrative astrocytomas.
- Can occur at any age but is especially common in children (intracranial) and young adults (spinal).
🧬 Aetiology & Pathology
- Myxopapillary ependymoma: The most common subtype, usually arising in the filum terminale and lumbar region; produces mucin.
- Other histological subtypes include classic and anaplastic (grade III).
- Ependymomas may lead to syringomyelia if located in the spinal cord, by disrupting CSF flow.
- Can arise anywhere in the ventricular system:
- Posterior fossa (4th ventricle): most common intracranial site in children → may obstruct CSF and cause hydrocephalus.
- Spinal cord: more common in adults.
🤝 Associations
- Neurofibromatosis Type 2 (NF2) – especially spinal ependymomas.
- Tuberous Sclerosis – less common but described association.
⚕️ Clinical Features
- Intracranial tumours:
- 💥 Headache (worse in the morning due to raised ICP).
- 🤢 Nausea/vomiting from hydrocephalus.
- 🚶 Gait disturbance / ataxia if cerebellar or posterior fossa involvement.
- Spinal tumours:
- ⚡ Sensory loss (often dissociated, if syrinx forms).
- 💪 Limb weakness from cord compression.
- 🩻 Back pain – a common early feature.
- Hydrocephalus may complicate both spinal and ventricular tumours due to CSF blockage.
🔎 Investigations
- MRI (preferred): Well-demarcated intraventricular or spinal canal mass, often with heterogeneous enhancement.
- CT: Can show calcification or mass effect, but less sensitive than MRI.
- Histology: Perivascular pseudorosettes are a classical microscopic feature.
💊 Management
- Total surgical resection – the mainstay of treatment where feasible.
- Radiotherapy / radiosurgery: Considered for residual disease or inoperable tumours, especially high-grade variants.
- Chemotherapy: Limited role, but sometimes used in recurrent or paediatric disease.
📌 Prognosis
- Myxopapillary (spinal) ependymomas usually have a good prognosis after complete resection.
- Anaplastic ependymomas (grade III) have a higher recurrence rate and worse survival.
- Recurrence is common if surgical resection is incomplete.
