Ependymoma
A tumor arising from cells associated with the fluid-filled spaces of the brain or spinal cord, where location, molecular type, and the extent of safe surgical removal all help guide treatment.
Quick Answer
An ependymoma is a tumor that develops from ependymal cells associated with the fluid-filled spaces of the brain and the central canal of the spinal cord. Ependymomas occur in both children and adults and may arise in the brain or spine. Today, diagnosis is based not only on how the tumor looks under the microscope, but also on where it arises and, for many ependymomas, its molecular features. Surgery is the primary treatment when feasible, with the goal of complete removal when that can be achieved safely. The extent of resection is an important factor in long-term tumor control, alongside the tumor’s location, molecular type, age of the patient, and whether disease has spread. Radiation is recommended in many cases depending on these factors and how much tumor remains after surgery.
Key Takeaways
- Ependymomas can arise in the brain or spinal cord and affect both children and adults.
- The 2021 WHO classification organizes ependymomas by anatomic site together with histologic and molecular features; several molecularly defined types are not assigned a traditional WHO grade.
- Symptoms depend on location and may result from pressure on the brain, spinal cord, nearby nerves, or blockage of cerebrospinal fluid flow.
- Surgery is the primary treatment when feasible, and achieving complete removal safely is an important predictor of long-term tumor control.
- Radiation may be recommended based on age, tumor location and molecular type, extent of resection, and whether disease has spread.
- Because ependymomas can spread through cerebrospinal fluid pathways, staging may include imaging of the entire brain and spine and, in selected cases, spinal fluid analysis.
- Long-term surveillance is important because recurrence can occur years after treatment.
At a Glance
| What it is | A tumor arising from ependymal cells associated with the fluid spaces of the brain and spinal cord |
|---|---|
| Location | Supratentorial brain, posterior fossa, or spine; anatomic site is part of the modern diagnosis |
| Who it affects | Children and adults; some tumor types occur more often in particular age groups |
| Classification | WHO CNS 2021 uses anatomic site with histologic and molecular features; several molecularly defined types are not assigned a traditional grade |
| Possible effects | Neurological symptoms or blocked cerebrospinal fluid flow, depending on location |
| Diagnosis | MRI, tissue analysis, and molecular characterization; craniospinal staging may be recommended |
| Key prognostic factors | Extent of safe surgical removal, tumor location and molecular type, patient age, and evidence of spread |
| Treatment | Surgery aimed at maximal safe removal, with radiation recommended in selected cases |
| Long-term care | Ongoing surveillance imaging because recurrence may occur years later |
Types and Locations of Ependymoma
Ependymoma is classified differently from many other brain and spinal tumors. The current World Health Organization classification considers where the tumor arises—above the tentorium, in the posterior fossa, or in the spine—together with its histologic and molecular features. Tumors that look similar under the microscope can behave differently depending on their location and molecular type.
Supratentorial Ependymoma
Arises in the cerebral hemispheres, above the tentorium. Molecularly defined types include ZFTA fusion-positive and YAP1 fusion-positive ependymomas. Identifying the molecular type can provide important information about how the tumor may behave.
Posterior Fossa Ependymoma
Arises at the back of the brain, often near the fourth ventricle and brainstem. The major molecular groups are PFA, seen mainly in younger children, and PFB, seen more often in older children and adults.
Spinal Ependymoma
Arises within the spinal cord and occurs most often in adults. Many have a definable surgical plane that can make complete removal possible. A rare MYCN-amplified type behaves more aggressively.
Myxopapillary Ependymoma
Usually arises near the lower end of the spinal canal and is classified as CNS WHO grade 2. When surgery is performed, preserving the tumor capsule and avoiding fragmentation when safely possible may reduce the risk of recurrence or spread.
Subependymoma
A slow-growing CNS WHO grade 1 tumor seen most often in adults. Small tumors that cause no symptoms may simply be monitored, while symptomatic tumors can often be treated successfully with surgery.
Why classification matters: For ependymoma, the diagnosis is more specific than a tumor name and grade alone. Location and molecular features help doctors understand how the tumor may behave and guide treatment planning and long-term follow-up.
Symptoms
Symptoms depend on where the ependymoma is located. In the brain, tumors near the ventricles can block the flow of cerebrospinal fluid and raise pressure, causing headaches, nausea, vomiting, and balance or coordination problems. In young children, increased pressure may instead appear as irritability, unsteadiness, or faster-than-expected head growth.
In the spine, ependymomas may cause back or neck pain, weakness, numbness, difficulty walking, or changes in bladder or bowel function. These symptoms can develop gradually and may initially resemble more common spine problems.
Symptoms that are severe, progressive, or associated with vomiting, drowsiness, new weakness, or bladder or bowel changes warrant prompt medical evaluation.
Common Signs and Symptoms
- Headache, nausea, or vomiting
- Balance or coordination problems
- Back or neck pain
- Weakness, numbness, or difficulty walking
- Changes in bladder or bowel function
When to Seek Care Promptly
- Severe or rapidly worsening headache with vomiting
- Increasing drowsiness or decline in alertness
- Rapidly worsening balance or coordination
- New or progressive weakness or numbness
- New bladder or bowel changes
Diagnosis
MRI with contrast is the primary imaging study and shows the tumor’s location, extent, and relationship to the brain, ventricles, spinal cord, and nearby structures. In spinal ependymoma, MRI may also show an associated syrinx, or fluid-filled cavity within the spinal cord.
Because ependymomas can spread through cerebrospinal fluid pathways, staging may include MRI of the entire brain and spine. When spinal staging imaging has not been completed before surgery, it is often delayed for roughly two to three weeks afterward so postoperative blood and other changes are not mistaken for tumor spread. Spinal fluid analysis may also be used in selected cases and is generally delayed after surgery for the same reason.
Why Molecular Testing Matters
A definitive diagnosis requires tumor tissue. A neuropathologist evaluates how the tumor looks under the microscope together with molecular testing appropriate to where it arose.
Supratentorial
Testing may identify ZFTA or YAP1 gene fusions.
Posterior Fossa
Tumors are classified into molecular groups such as PFA or PFB.
Spinal
Testing may look for MYCN amplification, which identifies a rare, more aggressive type.
In tumors that remain difficult to classify, DNA methylation profiling can sometimes provide additional information. Together, the tumor’s location, pathology, and molecular features establish the most precise diagnosis and help guide treatment.
Treatment
Treatment for an ependymoma depends on the tumor’s location, molecular characteristics, extent, the patient’s age, whether disease has spread, and its relationship to critical structures in the brain or spinal cord.
Surgery
Surgery is the primary treatment when feasible, with the goal of complete removal when that can be achieved safely. The extent of resection is an important factor in long-term tumor control, but it must always be balanced against the risk of neurological injury. When meaningful residual tumor remains and additional removal can be performed safely, a second operation may sometimes be considered before radiation.
In the posterior fossa, ependymomas can extend around the brainstem and toward cranial nerves that control swallowing, facial movement, and eye movement. In the spinal cord, many ependymomas have a clearer boundary from surrounding tissue than infiltrative spinal cord tumors, which can make complete removal more achievable. For myxopapillary ependymoma, avoiding capsular violation or fragmentation when safely possible is an important surgical consideration.
At Atlantic Brain and Spine, surgical planning may incorporate intraoperative neurophysiologic monitoring, stereotactic navigation, endoscopic assistance, and other technologies when appropriate for the tumor’s location. Care can draw on expertise in both brain and spinal tumor surgery.
Radiation & Additional Treatment
After surgery, the treatment plan is refined using the final pathologic and molecular diagnosis and postoperative imaging. Radiation is commonly used for many intracranial ependymomas and for tumors that cannot be completely removed, but recommendations vary by age, location, molecular type, extent of resection, and whether the tumor has spread. When disease is localized, radiation is generally directed to the tumor region; craniospinal radiation is reserved for patients with documented dissemination.
If a brain ependymoma has caused hydrocephalus, relieving pressure may be part of the initial treatment. In some patients, removing the tumor restores normal cerebrospinal fluid flow. Others may need an additional procedure to create or maintain a drainage pathway.
Long-Term Follow-Up
Ependymomas can recur years after treatment. Long-term surveillance therefore remains important. If a tumor returns, treatment may include repeat surgery, radiation or re-irradiation in selected cases, and other therapies based on the individual situation.
The Diagnosis-to-Treatment Pathway
1. Evaluation
History, neurological examination, and MRI identify the tumor, its location, and any effects on cerebrospinal fluid flow or neurological function.
2. Staging
Brain and spine imaging evaluate the full neuraxis for additional disease; spinal fluid analysis may be used in selected cases.
3. Surgical planning
The team determines the safest route and whether complete removal appears achievable without unacceptable neurological risk.
4. Surgery and tissue diagnosis
Surgery treats the tumor and provides tissue for definitive histologic and molecular diagnosis.
5. Molecular classification
Testing helps define the tumor type and provides information that can influence prognosis, radiation planning, and follow-up.
6. Additional treatment
Radiation is recommended when appropriate based on age, tumor type and location, extent of resection, and evidence of spread.
7. Long-term surveillance
Follow-up imaging continues for years because ependymomas can recur late.
Frequently Asked Questions
An ependymoma is a tumor arising from ependymal cells associated with the fluid-filled spaces of the brain and the central canal of the spinal cord. It can occur in the brain or spine and affects both children and adults.
The 2021 WHO classification uses anatomic site together with histologic and molecular features. Supratentorial, posterior fossa, and spinal ependymomas include distinct molecular types, while subependymoma is CNS WHO grade 1 and myxopapillary ependymoma is CNS WHO grade 2. Several molecularly defined ependymomas are not assigned a traditional WHO grade.
When an ependymoma can be removed safely, the amount of tumor remaining after surgery is an important factor in long-term tumor control. The goal is complete removal when feasible without causing unacceptable neurological injury.
Yes. Brain ependymomas near the ventricles can block cerebrospinal fluid flow and raise pressure inside the skull. Removing the tumor may restore normal flow, although some patients need an additional procedure to treat hydrocephalus.
Not everyone receives the same recommendation. Radiation decisions depend on age, tumor location and molecular type, how completely the tumor was removed, and whether there is evidence of spread. Your care team uses the final pathology and postoperative imaging to guide that decision.
Yes. Ependymomas can spread through cerebrospinal fluid pathways. For that reason, staging may include MRI of the entire brain and spine and, in selected cases, spinal fluid analysis.
They can. Recurrence may occur years after treatment, which is why long-term surveillance imaging is important. If an ependymoma returns, repeat surgery, radiation, or other treatment may be considered depending on the location and prior treatment.
Specialists Who Treat Ependymomas
Your care is provided by Atlantic Brain and Spine’s experienced brain and spine tumor specialists, with treatment tailored to the location and characteristics of the tumor.
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