A Guide To Ependymomas: What Patients Should Know

What Are Ependymomas?

Ependymomas are rare brain and spinal cord tumors that develop from ependymal cells — the specialized cells lining the ventricles and central canal, which help produce and circulate cerebrospinal fluid (CSF). They account for approximately 2–3% of all primary brain tumors and are less common than astrocytomas or the more aggressive medulloblastomas.

While medulloblastoma vs. ependymoma comparisons show that both can affect similar regions, ependymomas usually grow more slowly and respond differently to treatment. Unlike syringomyelia, which involves fluid-filled cavities, ependymomas form solid masses that can obstruct CSF flow and disrupt normal brain or spinal cord function.

Symptoms of Ependymomas

Recognising ependymoma symptoms early is crucial for timely intervention. The symptoms vary significantly depending on whether you're dealing with a brain tumour or a spinal cord tumour.

Brain-based ependymoma symptoms include:

• Persistent headaches, often worse in the morning
• Nausea and vomiting, particularly upon waking
• Vision problems, including blurred or double vision
• Seizures (abnormal electrical activity in the brain)
• Balance difficulties and coordination problems
• Confusion or personality changes

Spinal cord ependymoma symptoms present differently:

• Chronic back or neck pain lasting months
• Numbness or tingling in arms or legs
• Progressive weakness affecting mobility
• Loss of bladder or bowel control
• Difficulty walking or maintaining balance

Causes and Risk Factors

The exact causes of ependymomas are largely unknown, unlike syringomyelia, which can result from trauma or congenital abnormalities. Genetic factors may contribute, especially in hereditary conditions such as neurofibromatosis type 2 (NF2). However, most ependymomas occur sporadically, without identifiable environmental triggers.

The risk follows a bimodal distribution — peaking in children under 5 years and again in adults aged 30–40 years, and prior head or spine radiation may slightly increase risk. When comparing medulloblastoma vs. ependymoma, some risk features overlap, but they tend to affect different age groups.

According to the National Cancer Institute reports, ependymomas occur across all ages, with posterior fossa tumours more common in children and spinal ependymomas more common in adults, reflecting a strong age- and location-based pattern in disease presentation.

Types of Ependymomas

Understanding different ependymoma types helps clarify treatment approaches and prognosis. The World Health Organization (WHO) classifies ependymomas into three main grades:

Grade I Ependymomas:

• Myxopapillary ependymomas (typically spinal)
• Subependymomas (usually brain-based)
• Slow-growing with better outcomes

Grade II Ependymomas:

• Classic ependymomas affecting the brain and spine
• Moderate growth rate requiring careful monitoring
• Most common type in children

Grade III Ependymomas:

• Characterized by rapid growth
• Increased recurrence risk
• The need for aggressive multimodal therapy (surgery plus radiation)

Diagnosing Ependymomas

Diagnosing ependymomas requires a systematic approach combining clinical evaluation with advanced imaging techniques:

1. Initial neurological examination assessing reflexes, coordination, and cognitive function.
2. Detailed symptom history documenting ependymoma symptom progression.
3. Advanced imaging studies, including an MRI scan and potentially a CT scan.
4. Tissue sampling through biopsy for definitive ependymoma histology confirmation.
5. Cerebrospinal fluid (CSF) analysis to check for tumor cells or biochemical markers of spread.
6. Additional staging studies are determining the extent and spread.

Imaging and Diagnostic Methods for Detecting Ependymomas

Modern ependymoma radiology relies heavily on sophisticated imaging techniques that provide detailed visualisation of these complex tumours:

MRI Scan capabilities

• Superior soft tissue contrast for brain tumour detection
• Detailed ependymoma histology correlation through imaging
• Assessment of cerebrospinal fluid flow patterns
• Differentiation from conditions like syringomyelia

CT Scan applications:

• Initial emergency evaluation for acute symptoms
• Assessment of calcifications within tumours
• Post-surgical monitoring and follow-up imaging

Specialised imaging techniques:

• Functional MRI (fMRI) for mapping eloquent brain regions
• Diffusion tensor imaging evaluating white matter tracts
• Magnetic resonance spectroscopy (MRS) provides metabolic data that can help differentiate tumor grade or recurrence

Treatment Options for Ependymomas

Treatment for ependymomas differs from that for astrocytoma or medulloblastoma and needs specialised, individualised planning. Surgery is the primary treatment, aiming for complete tumour removal while preserving neurological function, usually followed by radiation therapy, especially for incomplete resections or higher-grade tumours.

Unlike syringomyelia, which is treated with drainage procedures, ependymomas require oncological management. Chemotherapy has a limited role in standard ependymoma treatment protocols compared with medulloblastoma, though ongoing trials are evaluating its benefit in recurrent cases, but newer targeted therapies may help in recurrent cases, guided by a multidisciplinary team.

Ependymoma Prognosis

Prognosis for ependymomas depends on tumour grade, location, patient age, and how much of the tumour can be removed. Complete surgical removal offers the best outlook, with five-year survival rates typically range between 65% and 85%, depending on tumor grade and extent of resection. Spinal cord ependymomas usually have a better prognosis than brain tumours because they’re easier to access surgically, and many ependymomas grow slowly compared with high-grade astrocytomas. Regular follow-up with MRI scans is essential to detect any recurrence early and tailor ongoing care to your individual risk and treatment response.

Managing Ependymomas in Children

Pediatric ependymomas require specialized care that balances tumor control with preservation of neurological, endocrine, and developmental function. Children may present differently, sometimes showing developmental delays, irritability, or regression rather than typical adult symptoms. Treatment often adapts radiation dose and timing to reduce long-term harm, and unlike adult astrocytoma or syringomyelia care, paediatric management must involve developmental specialists, school support, and family counselling. Lifelong follow-up with developmental, endocrine, and cognitive assessments is essential to detect and manage late treatment effects early.

Coping with Ependymomas

Living with an ependymoma diagnosis affects not only patients but entire families, requiring comprehensive support strategies:

Emotional support strategies:

• Connect with ependymoma support groups and online communities.
• Seek professional counseling or psychological support to manage anxiety, depression, or adjustment challenges.
• Maintain open communication with family and friends about your needs.

Practical management approaches:

• Organise medical appointments and maintain treatment records.
• Prepare questions before healthcare visits to maximise consultation time.
• Establish relationships with your multidisciplinary care team members.

Lifestyle modifications:

• Prioritise adequate rest and stress management techniques.
• Maintain physical activity as tolerated and medically appropriate.
• Focus on nutrition to support overall health during treatment.

Conclusion

Ependymomas require early diagnosis, multidisciplinary evaluation, and long-term follow-up to achieve the best possible outcomes. Understanding the tumour’s grade, location, and behaviour helps guide treatment choices and long-term monitoring, making accurate testing and regular imaging essential throughout the patient’s care journey.

Metropolis Healthcare supports this process with 4,000+ tests, full body checkups, and advanced speciality diagnostics, all backed by 10,000+ home collection touchpoints for unmatched convenience. With quick, accurate results and easy booking through the website, Metropolis ensures patients receive reliable, seamless diagnostic support at every stage.

FAQs

What are the early signs of ependymomas?

Early signs include persistent headaches, nausea, balance issues, back pain, or unexplained weakness. In children, early symptoms may include developmental delays or irritability.

Can ependymomas be treated without surgery?

Surgery is the primary treatment. In rare cases where surgery is unsafe, radiation therapy or targeted treatments may be considered. Observation is an option for small, asymptomatic tumours.

How long do ependymoma patients survive?

Survival depends on tumour grade and treatment success. Many patients with low-grade ependymomas live for years or even decades with proper treatment and monitoring.

What are the side effects of ependymoma treatment?

Several side effects of ependymomas include:

• Fatigue
• Hair loss (from radiation)
• Memory or concentration issues
• Balance problems
• Nausea or appetite changes

References

• https://www.nhs.uk/conditions/brain-tumours/
• https://www.cancerresearchuk.org/about-cancer/brain-tumours/types
• https://www.cancer.gov/rare-brain-spine-tumor/tumors/ependymoma
• https://my.clevelandclinic.org/health/diseases/23147-ependymoma
• https://www.mayoclinic.org/diseases-conditions/ependymoma/symptoms-causes/syc-20580744