What is an Ependymoma?
Ependymoma is a type of primary brain and spinal cord tumour that develops from cells associated with the lining of the brain’s ventricles and the central canal of the spinal cord.
Doctors classify ependymoma based on both its location in the central nervous system (CNS) and its molecular features. It can affect both children and adults. However, tumours that arise within the brain, particularly in the posterior fossa, occur more commonly in children, while spinal ependymomas are more common in adults.
Types of Ependymoma
Ependymoma is not a single disease. Doctors now classify ependymoma based on where it develops in the CNS, along with its molecular and microscopic features. These differences help guide diagnosis, prognosis, and treatment.
In addition to the main groups below, doctors also recognise two distinct tumour types based on biological and clinical features: myxopapillary ependymoma and subependymoma.
This type develops in the upper part of the brain and occurs more commonly in children than adults. The two main molecular subtypes include ZFTA fusion-positive ependymomas, which is the most common, and YAP1 fusion-positive ependymomas.
These tumours develop in the back part of the brain and also occur more frequently in children than adults. They are divided into two main groups: Posterior Fossa Group A (PFA) and Posterior Fossa Group B (PFB).
PFA tumours are more common in young children and are generally associated with poorer outcomes, while PFB tumours are more often seen in older children and adults and tend to have more favourable outcomes.
Spinal ependymomas occur more commonly in adults than in children. Some cases are associated with NF2 gene alterations. A subset of spinal ependymomas has also been found to carry MYCN alterations, which are associated with more aggressive disease.
Ependymoma Symptoms
Symptoms of ependymoma depend strongly on where the tumour develops in the brain or spinal cord. Symptoms may develop gradually and sometimes only become noticeable once the tumour increases pressure inside the skull.
Ependymomas that arise near the brain’s ventricular system can block the flow of cerebrospinal fluid (CSF). This blockage can lead to fluid buildup and increased intracranial pressure.
Common symptoms include:
- Headache
- Dizziness
- Nausea and vomiting
- Double vision (diplopia)
- Seizures
If the tumour presses on nearby brain structures, it can disrupt their normal function and cause:
- Balance problems
- Difficulty walking
When the tumour affects the brainstem, symptoms may include:
- Hearing difficulties
- Difficulty swallowing
Spinal ependymoma, symptoms may include:
- Weakness in the limbs
- Numbness, weakness, or difficulty walking
- Pain
- Bladder dysfunction
How is Ependymoma Diagnosed?
Doctors investigate suspected ependymoma using imaging techniques such as MRI and CT scans. CT scans can help detect calcifications or changes in nearby bone structures associated with some ependymomas. However, MRI provides much clearer detail of the tumour, especially when doctors use a contrast agent such as gadolinium to help distinguish tumour tissue from normal brain tissue.
MRI of the brain and spinal cord is therefore important for assessing the tumour and determining whether it has spread through the central nervous system. Advanced MRI techniques, including perfusion imaging and MR spectroscopy, may also provide additional information about tumour activity and characteristics.
If doctors suspect that the tumour has spread through the cerebrospinal fluid (CSF), they may recommend CSF cytology testing. This test examines the fluid for tumour cells that may have travelled to other areas of the central nervous system.
After surgery or biopsy, pathologists examine tumour tissue under the microscope and may perform molecular testing to classify the ependymoma subtype.
Living with Ependymoma
Life after ependymoma treatment can be challenging for both patients and their caregivers. Even after successful treatment, your multidisciplinary team may recommend regular MRI scans to monitor for tumour recurrence and detect changes early as possible. This ongoing follow-up can create anxiety and uncertainty about the future. Although scan frequency often decreases over time, many patients still require long-term monitoring.
Your healthcare team may also schedule regular visits after treatment to monitor both early and late side effects. Some patients find it difficult to return to daily activities, including sports and exercise. However, incorporating physical activity into daily routines can improve balance, reduce pain, and support social interaction and overall well-being.
Treatment can also affect emotional and cognitive health. Some patients experience fatigue, depression, or difficulties with concentration and memory. Children treated with radiotherapy or certain chemotherapy drugs may also develop hearing problems as a long-term side effect of treatment. For this reason, childhood survivors often require close long-term medical follow-up as they transition into adulthood.
Ependymoma Treatment and Management Options
Treatment for ependymoma mainly involves surgery and radiotherapy. Chemotherapy is used less commonly than surgery or radiotherapy, but it may be considered in young children, recurrent disease, or when standard treatments are not suitable. They may also use chemotherapy if the tumour recurs after initial treatment.
Whenever possible, surgeons aim to completely remove the tumour, as complete resection associated with improved tumour control and survival outcomes.
However, complete removal is not always safe or feasible. Tumours located near critical or eloquent areas of the brain may place important functions at risk during surgery.
Surgery may also be unsafe when the tumour is difficult to access or when the patient’s age or overall condition increases surgical risk.
In some cases where surgery is not possible or safe, radiotherapy may be used as the main treatment. Modern radiotherapy techniques may include highly targeted approaches such as intensity-modulated radiotherapy (IMRT) or proton therapy, which aim to reduce radiation exposure to surrounding healthy tissue.
Your oncology team will determine the most appropriate approach based on the tumour’s location, extent of spread, molecular subtype, and your overall health.
Ependymoma can affect both children and adults and often involves surgery and ongoing monitoring for recurrence. For carers, this can mean managing recovery, long-term side effects, and the uncertainty of follow-up care.
The impact can be physical, emotional, and financial, particularly where treatment is intensive or ongoing.
Find support information and resources to help you navigate these challenges, while our National Advocacy Service can support you in accessing care, understanding your options, and advocating for your loved one.
References
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- Butenschoen, V. M., Gloßner, T., Hostettler, I., Meyer, B., & Wostrack, M. (2021). Return to work, activities of daily living and sports after spinal ependymoma resection: a monocenter experience with long-term follow-up. https://doi.org/10.21203/rs.3.rs-1053627/v1
- Coltin, H., Pequeno, P., Liu, N., Tsang, D. S., Gupta, S., Taylor, M. D., Bouffet, E., Ramaswamy, V., & Nathan, P. C. (2024, 2024/11/01). The medical and functional burden of surviving childhood ependymoma: A population-based study in Ontario, Canada. Pediatric Blood & Cancer, 71(11), e31275. https://doi.org/https://doi.org/10.1002/pbc.31275
- Jünger, S. T., Timmermann, B., & Pietsch, T. (2021, 2021/08/01). Pediatric ependymoma: an overview of a complex disease. Child’s Nervous System, 37(8), 2451-2463. https://doi.org/10.1007/s00381-021-05207-7
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