ConditionCancer
Ependymoma
An ependymoma is a tumor of the brain or spinal cord that grows from ependymal cells, which line the fluid-filled ventricles and the central canal of the spinal cord. In children it most often arises in the back of the brain (posterior fossa), causing headaches, vomiting and balance problems from blocked fluid flow. In adults it is more common in the spinal cord, causing back pain, numbness and weakness. The main treatment is complete surgical removal, often followed by radiotherapy.
Also called: Ependymal tumor, CNS tumor, spinal ependymoma, posterior fossa ependymoma
At a glance
- What it is
- A glial tumor arising from cells lining the brain's ventricles or the spinal canal.
- Who gets it
- Children (often under 5, posterior fossa) and adults (often spinal cord).
- Symptoms
- Headaches, morning vomiting, unsteadiness, neck pain in brain tumors. Back pain, numbness and weakness in spinal tumors.
- Diagnosis
- MRI of the brain and whole spine, then surgery and tissue analysis, including molecular type.
- Treatment
- Maximal safe surgical removal, focal radiotherapy, and sometimes chemotherapy.
When to get help
Call 911 or go to the ER
- Sudden severe headache, repeated vomiting, drowsiness or confusion (possible hydrocephalus)
- A seizure, especially the first one
- Sudden leg weakness, loss of bladder or bowel control, or numbness in the saddle area
See a doctor within a day or two
- Morning headaches with vomiting that are getting worse over days to weeks, especially in a child
- New unsteadiness, clumsiness, double vision or head tilt in a child
- An enlarging head size or bulging soft spot in a baby
Book a routine appointment
- Persistent back or neck pain, especially at night, with numbness or tingling
- Gradual changes in walking or bladder control
What an ependymoma is
Ependymal cells line the ventricles, the cavities in the brain filled with cerebrospinal fluid (CSF), and the central canal of the spinal cord. Ependymomas arising from these cells can block CSF flow, causing hydrocephalus and raised pressure in the head, or press on the spinal cord (spinal cord compression).
The 2021 WHO classification groups ependymomas by location and molecular features, which predict behavior better than appearance under the microscope:
| Location | Main molecular types | Typical patients and behavior |
|---|---|---|
| Supratentorial (upper brain) | ZFTA fusion-positive, YAP1 fusion-positive | Children and adults. ZFTA type can be aggressive. YAP1 type often favorable |
| Posterior fossa (back of brain) | PFA and PFB groups | PFA mostly in infants and young children, higher risk. PFB in teens and adults, more favorable |
| Spinal | Spinal ependymoma, MYCN-amplified, myxopapillary | Mostly adults. Myxopapillary type at the base of the spine, often slow-growing |
| Any | Subependymoma | Slow-growing, often found incidentally in adults |
Tumors are graded 1 to 3 by WHO, with grade 3 being the most aggressive.
Causes and risk factors
The cause is usually unknown. Neurofibromatosis type 2 (now called NF2-related schwannomatosis) increases the risk of spinal ependymomas. Most ependymomas are not inherited.
Symptoms
Brain ependymomas
- headaches, often worse in the morning or on lying down
- nausea and vomiting, often on waking
- unsteadiness, clumsiness and dizziness (balance problems)
- double vision or a head tilt
- neck pain or stiffness
- irritability, sleepiness, loss of milestones in young children
- enlarging head and bulging soft spot in babies
- seizures with supratentorial tumors
Spinal ependymomas
- back or neck pain, often worse at night
- numbness, tingling or weakness in the arms or legs
- difficulty walking
- bladder or bowel changes
- myxopapillary tumors may cause low back pain and sciatica-like leg pain
Diagnosis
- neurological examination
- MRI of the brain and entire spine with contrast: shows the tumor and checks whether it has spread through the CSF
- lumbar puncture: CSF analysis for tumor cells, usually done after surgery when safe
- biopsy or surgical removal: confirms the diagnosis, with molecular testing to identify the subtype
Treatment
Treatment is planned by a specialist neuro-oncology team, ideally in a center experienced with ependymoma.
Surgery
The most important factor for outcome is how completely the tumor is removed (gross total resection). Surgeons use navigation, intraoperative imaging and nerve monitoring to remove as much as safely possible. A second operation may be recommended if a postoperative MRI shows residual tumor that can be removed. A shunt or endoscopic third ventriculostomy may be needed for hydrocephalus.
Radiotherapy
Focused radiotherapy to the tumor bed is standard for most intracranial grade 2 and 3 ependymomas, including in children over about 1 year. Proton therapy can reduce radiation to surrounding healthy brain in children. Craniospinal radiation is used when the tumor has spread.
Chemotherapy
Chemotherapy has a more limited role. It may be used in very young children to delay radiation, for residual tumor before second surgery, or for recurrence. Clinical trials are testing targeted treatments based on molecular subtype.
Spinal and myxopapillary ependymomas
Complete removal can be curative. Radiotherapy may follow incomplete removal or high-grade tumors.
Follow-up and long-term effects
Regular MRI scans continue for many years, because ependymomas can recur late. Long-term effects of treatment, especially in young children, can include:
- learning and memory difficulties
- hearing loss
- hormone problems from radiation to the pituitary region
- growth problems
- balance and coordination difficulties
- risk of second tumors after radiation
Survivorship clinics provide monitoring, rehabilitation and educational support.
Outlook
Outcomes vary widely by location, molecular type, age and completeness of surgery. Many spinal ependymomas and completely removed favorable-group tumors have excellent long-term control. Posterior fossa group A tumors in young children and tumors that cannot be fully removed have higher recurrence rates. Your team can give a personalized outlook based on these factors.
Frequently asked questions
What is an ependymoma?
An ependymoma is a tumor of the central nervous system that grows from cells lining the fluid spaces of the brain and the central canal of the spinal cord. It can occur in children and adults.
What are the symptoms of an ependymoma in children?
Common symptoms include morning headaches, vomiting, unsteadiness, clumsiness, double vision, neck pain, irritability and, in babies, an enlarging head. These come mostly from blocked fluid flow and raised pressure in the head.
Is ependymoma cancerous?
Ependymomas are primary central nervous system tumors. Some are slow-growing (grade 1), while others are more aggressive (grade 2 or 3). They rarely spread outside the brain and spine but can spread through the spinal fluid.
How is ependymoma treated?
The main treatment is surgery to remove as much of the tumor as safely possible, usually followed by focused radiotherapy. Chemotherapy is used in some situations. Regular MRI follow-up checks for recurrence.
What is the survival rate for ependymoma?
Survival depends on the tumor's location, molecular subtype, grade, patient age and whether the tumor was completely removed. Many patients, especially with completely removed or spinal tumors, have long-term survival. Your care team can give the most accurate estimate.
Sources
This guide was written from current patient and clinical guidance, including:
- National Cancer Institute: Childhood ependymoma treatment (PDQ); Adult central nervous system tumors treatment (PDQ)
- World Health Organization: Classification of Tumours of the Central Nervous System, 5th edition (2021)
- American Brain Tumor Association: Ependymoma
- CERN Foundation: Ependymoma information
- American Cancer Society: Brain and spinal cord tumors in children