Ependymoma: Symptoms, Causes, and Treatment

Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026

Ependymoma begins in the cells lining the cerebrospinal fluid passageways and can form in the brain or the spinal cord.

Quick answer

Ependymoma is a tumor that forms in the brain or spinal cord, starting in the ependymal cells that line the passageways carrying cerebrospinal fluid. It most often occurs in young children. Symptoms include headaches, seizures and vomiting, or back pain and leg weakness. Surgery is the primary treatment, followed by radiation or other therapies if needed. [1] [2]

What the research says

According to PubMed:

• Adding chemotherapy after radiation did not clearly improve outcomes in children. In the Children's Oncology Group phase 3 trial ACNS0831, 325 patients with a complete or near-total resection (or complete response to induction therapy) were randomized. Five-year event-free survival was 63.7% with radiation alone versus 69.2% with radiation plus chemotherapy (HR 0.866; 1-sided log-rank P = .299), and five-year overall survival was 86.9% versus 88.3% (Smith et al., Neuro-Oncology, 2026; DOI).

• How much tumor is removed matters. In the earlier COG trial ACNS0121 (356 patients aged 1 to 21), five-year event-free survival was 68.5% (95% CI 62.8-74.2%) after near-total or gross total resection followed by conformal radiation, compared with 37.2% (95% CI 24.8-49.6%) after subtotal resection (Merchant et al., J Clin Oncol, 2019; DOI).

• In spinal cord tumors, complete removal improved survival. A systematic review of 57 articles covering 3,022 patients with intramedullary (spinal cord) ependymoma and astrocytoma found that gross total resection improved overall survival (HR 1.68; p < 0.01), and 79% of patients had stable or improved neurological function six months after surgery (Hamilton et al., J Clin Neurosci, 2019; DOI).

Study details are from PubMed abstracts.

What is an ependymoma?

Ependymoma is a growth of cells that forms in the brain or spinal cord. The cells form a mass called a tumor. It begins in the ependymal cells, the cells that line the passageways that carry cerebrospinal fluid, the fluid that surrounds and protects the brain and spinal cord. [1]

There are different types of ependymomas. Some grow slowly and are not considered cancerous; these are called benign tumors, and a benign ependymoma may grow large enough to press on nearby tissue. Others grow more quickly and are considered cancerous. These malignant tumors may invade and destroy nearby tissue, and cancerous cells can also break away and spread within the brain or spinal cord. [1]

Feature	Benign (noncancerous)	Malignant (cancerous)
Growth speed	Grows slowly	Grows more quickly
Behavior	May press on nearby tissue	May invade and destroy nearby tissue
Spread	Not considered cancerous	Cells can break away and spread within the brain or spinal cord

Symptoms depend on location

Ependymoma signs and symptoms depend on where the tumor forms, in the brain or in the spinal cord. The symptoms follow the area the tumor affects. [1]

Ependymoma symptoms by location. Children most often have headaches and seizures.

Symptoms of ependymoma in the brain

• Blurry vision

• Confusion

• Headaches

• Irritability

• Nausea

• Seizures

• Vomiting

Symptoms of ependymoma in the spinal cord

• Bowel changes

• Loss of balance

• Pain in the neck or back

• Trouble walking

• Weakness in the legs

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you, such as persistent headaches, seizures, blurry vision, unexplained back pain, or trouble walking. [1]

What causes ependymoma?

It is not clear what causes ependymoma. The tumor starts when cells in the brain or spinal cord develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA tells cells to grow and multiply at a set rate and to die at a set time. In tumor cells, the DNA changes give different instructions: grow and multiply quickly, and keep living when healthy cells would die. This causes too many cells, which form the tumor. [1]

Risk factors

Risk factor	What it means
Young age	Ependymoma can happen at any age, but happens most often in children
Inherited syndromes	Some ependymomas are linked to genetic conditions passed down in families, such as neurofibromatosis type 2 and Li-Fraumeni syndrome

Ependymoma in children versus adults

Ependymoma can occur at any age, but most often happens in young children, who may have headaches and seizures. In adults it is more likely to form in the spinal cord, where it may cause weakness in the part of the body controlled by the affected nerves. [1]

How ependymoma is diagnosed

A healthcare team uses a combination of exams and tests. [2]

From exam to treatment: neurological exam, MRI, spinal tap, and surgery, with radiation, radiosurgery, chemotherapy or targeted therapy if needed.

Step	Test or procedure	What it reveals
1	Neurological exam	Vision, hearing, balance, coordination, strength and reflexes, which give clues about the affected area
2	Imaging (MRI)	Location and size of the ependymoma; often of both the brain and spinal cord
3	Cerebrospinal fluid testing (spinal tap)	Whether tumor cells are present in the spinal fluid
4	Surgery and lab testing	The removed tumor is tested to confirm the diagnosis and identify the cell types involved

A lumbar puncture, or spinal tap, involves inserting a needle between two bones in the lower spine to draw out fluid from around the spinal cord. Once the tumor is removed, special lab tests tell the healthcare team about the types of cells involved, and this information may guide treatment decisions. [2]

Treatment options

Surgery

Neurosurgeons work to remove as much of the ependymoma as possible, ideally the entire tumor. Sometimes the tumor sits near sensitive brain or spinal tissue that makes complete removal too risky. If the entire tumor is removed, additional treatment may not be needed. If some tumor remains, the neurosurgeon may recommend another operation to remove the rest. [2] European guidelines likewise describe surgery as the crucial initial treatment in children and adults, and gross total resection as the mainstay of treatment for spinal ependymomas (EANO guidelines).

Radiation therapy

Radiation therapy uses powerful energy beams, such as X-rays or protons, to kill tumor cells. It may be recommended after surgery to help prevent cancerous tumors from coming back, or when the tumor could not be removed completely. Focused techniques, including conformal radiation therapy, intensity-modulated radiation therapy, and proton therapy, aim to spare nearby healthy cells. [2]

Stereotactic radiosurgery

Radiosurgery is an intense form of radiation treatment. Beams are aimed from many angles at the tumor; each beam is not very powerful, but the point where they meet receives a very large dose that kills the tumor cells. [2]

Chemotherapy

Chemotherapy uses medicines to kill tumor cells. It is not often used for ependymoma, but it might be an option in certain situations, such as when the tumor grows back despite surgery and radiation. [2]

Targeted therapy

Targeted therapy uses medicines that attack specific chemicals in tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die. It might be an option for an ependymoma that comes back after treatment. [2]

Clinical trials

Clinical trials are studies of new treatments. They offer a chance to try the latest options, although the risk of side effects may not be known. Talk with your healthcare team if you are interested. [2]

Treatment	How it works	When it is used
Surgery	Neurosurgeons remove as much of the tumor as possible	Primary treatment; may be the only treatment needed
Radiation therapy	Energy beams (X-rays, protons) kill tumor cells	After surgery for cancerous tumors, or when removal is incomplete
Radiosurgery	Many weak beams meet at one point to deliver a large dose	A focused form of radiation treatment
Chemotherapy	Medicines kill tumor cells	Not common; an option when the tumor grows back
Targeted therapy	Medicines block specific chemicals in tumor cells	An option for a tumor that comes back after treatment
Clinical trials	Studies of new treatments	When the latest options are of interest to the patient

Preparing for your appointment

If a healthcare professional thinks you might have ependymoma, you may be referred to a neurologist, a doctor who specializes in diseases of the nervous system, and to an oncologist if a cancer diagnosis is made. Because appointments can be brief, preparation helps: ask whether anything must be done in advance, write down all symptoms (including ones that seem unrelated), list all medicines, vitamins, and supplements with doses, note major stresses or recent life changes, bring a family member or friend, and write down your questions from most to least important.

Useful questions include: Do I have an ependymoma? Is it cancerous? What is its grade? Has it spread? Will I need more tests? What are the treatment options and the side effects of each? How will treatment affect my daily life? Should I see a specialist? Be ready to answer when your symptoms began, whether they are continuous or occasional, how severe they are, and what improves or worsens them.

Conclusion

Ependymoma is a brain or spinal cord tumor that begins in the cells lining the cerebrospinal fluid passageways. Symptoms depend on location: headaches, seizures, and vision changes point to the brain, while back pain, walking trouble, and leg weakness point to the spinal cord. Surgery to remove as much of the tumor as possible is the primary treatment, and radiation, radiosurgery, chemotherapy, or targeted therapy may follow when the tumor grows quickly or cannot be fully removed. [1] [2]

Next steps: If you or your child have worrying symptoms such as persistent headaches, seizures, blurry vision, unexplained back pain, or trouble walking, make an appointment with a healthcare professional. If ependymoma is suspected, ask for a referral to a neurologist or oncologist, and bring a written list of symptoms, medicines, and questions.

Frequently asked questions

What is an ependymoma?

Ependymoma is a tumor that forms in the brain or spinal cord. It begins in ependymal cells, which line the passageways that carry cerebrospinal fluid, the fluid that surrounds and protects the brain and spinal cord. [1]

Is an ependymoma cancerous?

An ependymoma can be. Some ependymomas grow slowly and are benign, meaning they are not considered cancerous. Others grow quickly, are malignant (cancerous), and can invade nearby tissue or spread within the brain or spinal cord. [1]

What are the symptoms of ependymoma?

Symptoms depend on the tumor's location. Brain ependymomas can cause blurry vision, confusion, headaches, irritability, nausea, seizures, and vomiting. Spinal cord ependymomas can cause bowel changes, loss of balance, neck or back pain, trouble walking, and weakness in the legs. [1]

How is ependymoma treated?

Surgery is the primary treatment, with the goal of removing as much of the tumor as possible. If the tumor grows quickly or cannot be removed completely, additional treatments may be recommended, including radiation therapy, stereotactic radiosurgery, chemotherapy, or targeted therapy. [2]

Who is most likely to get ependymoma?

Ependymoma can occur at any age, but it happens most often in young children, who often experience headaches and seizures. In adults, ependymoma is more likely to form in the spinal cord. Risk is also higher in people with certain inherited syndromes, such as neurofibromatosis type 2 and Li-Fraumeni syndrome. [1]

This article is for general education and is not a substitute for professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare professional about your individual situation.

References

1. Mayo Clinic Staff. Ependymoma: Symptoms and causes. Mayo Clinic. March 22, 2025. https://www.mayoclinic.org/diseases-conditions/ependymoma/symptoms-causes/syc-20580744

2. Mayo Clinic Staff. Ependymoma: Diagnosis and treatment. Mayo Clinic. March 22, 2025. https://www.mayoclinic.org/diseases-conditions/ependymoma/diagnosis-treatment/drc-20580745

3. Smith AA, Onar-Thomas A, Lensing S, et al. Phase 3 randomized trial of postirradiation chemotherapy in patients with newly diagnosed ependymoma: A report from the Children's Oncology Group. Neuro Oncol. 2026;28(5):1286-1297. PMID 41423745. https://doi.org/10.1093/neuonc/noaf285

4. Merchant TE, Bendel AE, Sabin ND, et al. Conformal Radiation Therapy for Pediatric Ependymoma, Chemotherapy for Incompletely Resected Ependymoma, and Observation for Completely Resected, Supratentorial Ependymoma. J Clin Oncol. 2019;37(12):974-983. PMID 30811284. https://doi.org/10.1200/JCO.18.01765

5. Hamilton KR, Lee SS, Urquhart JC, Jonker BP. A systematic review of outcome in intramedullary ependymoma and astrocytoma. J Clin Neurosci. 2019;63:168-175. PMID 30833131. https://doi.org/10.1016/j.jocn.2019.02.001

6. Rudà R, Reifenberger G, Frappaz D, et al. EANO guidelines for the diagnosis and treatment of ependymal tumors. Neuro Oncol. 2018;20(4):445-456. PMID 29194500. https://doi.org/10.1093/neuonc/nox166