Ependymoma

A tumor that starts in the lining of the fluid spaces of the brain and spine. Where it grows, and what the grade and the gene result mean. Why surgery matters so much. And why the scans go on for years.

The short version

An ependymoma is a tumor that starts in the lining of the fluid spaces in the brain and spine. In children it is most often low at the back of the brain. In adults it is more often in the spinal cord. Surgery is the main treatment, and how much of it comes out matters a great deal. Some ependymomas can be cured that way, when all of it can be taken out. It can also come back, even years later, so the scans go on for a long time.

What is an ependymoma?

Your brain has spaces inside it that are filled with fluid, and a thin channel of the same fluid runs down the middle of your spinal cord. Those spaces are lined with cells called ependymal cells. An ependymoma starts in that lining.

It belongs to the glioma family, in a group of its own. Many gliomas grow out into the brain around them. An ependymoma usually does not. That difference is a big part of why surgery matters so much here.

It is most common in children under 5. It can happen at any age, and adults get it too.

One name, several kinds

Doctors now name an ependymoma by where it is and by gene results from the tumor. Two that look alike under a microscope can behave very differently, so both parts of the name matter. The section on your report lists the names.

Is it cancer? What does its grade mean?

Grade 3 is malignant, which means cancer. Grade 2 is called low grade, which is about how fast it grows, not about how serious it is. A grade 2 ependymoma is still treated with surgery, and it is still followed with scans for years, because it can come back.

The grade is only part of the picture.

Most ependymomas are grade 2 or grade 3. Grade 2 tends to grow more slowly, and grade 3 tends to grow faster.

For this tumor, the grade does not always match how it behaves. That is why your team also looks at where it is and at its gene result.

Two kinds have a grade set by their name. Myxopapillary ependymoma, low in the spine, is grade 2. Subependymoma is grade 1. It is often found by chance in older adults, and it often needs no treatment unless it is causing symptoms or growing.

Grade 1 is the slowest-growing kind. If your report says grade 1, or carries a name with no grade beside it, that is the whole answer, not a gap in it. Ask your team what your own report means.

Can it be cured?

Some ependymomas can be cured by surgery, when all of it can be taken out. That is not always possible, because of where it sits. When some has to be left behind, it is much harder to cure.

That is a hard sentence, and it is here on purpose. It is the reason your team puts so much into the first operation, and sometimes offers a second one.

It can also come back after treatment that went well, sometimes many years later. That is why the scans go on for so long. It does not mean anybody got anything wrong.

You may have read that a glioma of grade 2 or above cannot be cured. That is about the gliomas that grow out into the brain around them. An ependymoma usually does not grow that way, so that sentence was not written about this tumor.

Where does it grow, and why does it cause these symptoms?

It grows where the fluid spaces are, and where it sits decides what you notice.

• Low at the back of the brain. Doctors call this part the posterior fossa. It holds the cerebellum, which helps with balance, and the brain stem. Most ependymomas in children are here.
• Higher up in the brain. Here it is more likely to cause seizures, or weakness on one side of the body. Your report may call this supratentorial.
• In the spinal cord. Most ependymomas in adults are here. The kind called myxopapillary grows at the very bottom end of the cord.

Because it grows in the fluid spaces, it can block the fluid. Many of the symptoms come from that. The section below explains what it does.

Most people are told what they have long before anyone explains what it is doing. These are the ways a tumor in the brain causes symptoms. They overlap, and most people have more than one going on at once. Which of them apply to you depends on what your tumor is and where it sits.

It takes up room. The skull is a closed box. It cannot stretch. So anything new inside it has to push on something. The tumor and any swelling around it together take up space and press on nearby parts of the brain.

It can make the brain around it swell. This one surprises people, and it is worth understanding. Fluid builds up in the brain tissue near the tumor. That swelling plays a major part in deciding what symptoms you get. Some of what you are feeling may not be the tumor itself. It may be the swelling around it. That is why medicines that bring swelling down can change how somebody feels, without the tumor being any different than it was.

Pressure can build up inside the head. When the pressure inside the skull rises, there is a pattern doctors watch for: headaches that are worse in the morning, feeling sick to your stomach and throwing up, odd eye movements, seizures, and being confused or very drowsy. Not everybody with raised pressure has all of it, and some people have almost none of it. If this pattern is new for you, it is worth a phone call rather than a wait. If you have a shunt, it means getting help right away.

It can set off seizures. A tumor and the irritated brain around it can upset the normal electrical activity of that part of the brain. For some people a seizure is the first sign that anything is wrong.

It can block the flow of fluid. The brain makes a fluid that circulates around it and drains away. A tumor sitting in the wrong place can block that flow, so the fluid backs up and the pressure goes up. This is more likely with tumors near the narrow channels the fluid passes through. It can be treated, and the usual treatment is a shunt. That is a thin tube that carries the fluid to another part of the body, where it is absorbed. It is the word your team will use. Shunts and hydrocephalus covers what having one is like, and the signs it has stopped working.

Why your symptoms are the ones you have

The short rule: the symptom tells you where, the scan tells you what. A tumor disturbs whatever job the brain does in that spot, so where it sits decides what you notice.

There is one exception, and it is the fluid. When the flow is blocked, what you notice is the pressure rather than the spot: headaches worse in the morning, throwing up, double vision, and feeling unsteady. Those signs are much the same wherever the blockage sits, so they tell you the pressure is up, not where the tumor is. If that is your pattern, the part above called It can block the flow of fluid is the one about you, and it says what can be done. If the pattern is new, the phone call above applies.

• Front of the brain. Personality, behavior, planning, motivation, and on one side, producing speech.
• Side of the brain, near the temple. Seizures, memory, and understanding what people say.
• Upper back part of the brain. Sensation, knowing where your body is in space, reading, writing and arithmetic.
• Back of the brain. Vision, and losing part of your field of view.
• Cerebellum, low at the back. Balance, coordination and walking.
• Brainstem. Double vision, facial weakness, and trouble swallowing.
• Deep in the middle, near the spaces the fluid runs through. A tumor here can block the flow, so what you notice is often the pressure rising rather than anything that points to the spot.
• The floor of the skull, below and behind the ear. Hearing, balance, and the nerves to the face. So hearing loss or ringing in one ear, dizziness, and numbness or weakness down one side of the face. Your team may call the whole floor of the skull the skull base. This spot is one part of it.
• Behind the eyes, at the base of the brain. Vision, and the hormones that set energy, weight, thirst and periods. In a child, growth and puberty too. Side vision is what usually goes first, so things start disappearing at the edge. Your team may call this the pituitary or the sellar region.

If something on this list matches what you have noticed, it is worth writing down and taking to your next appointment. Your team can tell you whether it fits where your tumor actually is.

This list is about what the place explains. The other half of the question is what the place changes: whether an operation is offered, how much of it can come out, and when the place makes something urgent on its own. Where your tumor is, and what that changes is that half.

What symptoms does it cause?

It depends on where the tumor is.

Low at the back of the brain, most symptoms come from the blocked fluid:

• Headaches, often worst on waking up.
• Throwing up, often in the morning.
• Trouble with balance or walking.
• Feeling dizzy, or eyes that flick from side to side.

New or worse trouble with walking or balance is a same-day call, or a right-away call if there is a shunt, or if the tumor is in the spinal cord or has spread to the spine.

In a baby, the head can grow bigger than it should, the soft spot on top of the head can bulge, and the baby can be more irritable than usual. If you see that in a baby with this tumor, call your team the same day, or right away if there is a shunt.

Higher up in the brain: seizures, weakness on one side, trouble with speech, or changes in vision.

In the spinal cord: back or neck pain, numbness or weakness in the arms or legs, and trouble with the bladder or bowel. Low in the spine, it can cause numbness in the area you sit on. For a tumor in the spinal cord, or one that has spread to the spine, those signs have their own rule, at the end of the section on when to call for help, below.

When to call for help right now

Some things should not wait for the next appointment.

Call an ambulance for any of these. Call 911, or your local emergency number:

• A first ever seizure.
• A seizure that lasts more than five minutes.
• One seizure right after another, with no waking up in between.
• Someone who cannot be woken.
• Trouble breathing, or someone who seems to be choking.
• Suddenly not being able to speak, move one side, or see.

A seizure like the ones you usually have, that stops on its own, is not an ambulance call. What to do during a seizure draws the line.

Call your team the same day for any of these:

• A headache much worse than usual, or one that wakes you from sleep.
• Throwing up again and again.
• New weakness in the face, an arm or a leg.
• New trouble speaking, or understanding what people say.
• New confusion.
• Sleeping much more than being awake.
• A sudden change in your vision.

If you are having chemotherapy, a fever is its own rule. It means calling your team right away, at any hour, not waiting for the morning. An infection during chemotherapy can get dangerous fast, and it is treatable when it is caught early. The fever rule explains why, and what number your team will give you.

In the weeks after brain surgery, a fever is its own rule too, especially with a stiff neck or a bad headache. What comes after a craniotomy has the rest of that list.

If you have a shunt, the signs it has stopped working are their own rule. For you, everything on the same-day list above is a right-away call instead, and so are a few other signs. Get help right away, at any hour: call your team, or go to the emergency department. Signs a shunt is not working has the full list.

If you are not sure whether to call, call. Nobody on that team minds a phone call that turns out to be nothing.

Same day means today, not the next clinic appointment. Ask your team now for the after-hours number, and put it somewhere you will find it at two in the morning rather than somewhere sensible.

What to do during a seizure has the step-by-step, and getting help now is the short version to keep on a phone.

If the tumor is in the spinal cord, or has spread to the spine, new signs from the cord are their own rule. For you, an arm or a leg that is newly weak, or weaker than it was, is a right-away call, not a same-day one.

So are new trouble walking, new numbness or tingling, new or worse pain in the back or neck, and new trouble controlling the bladder or bowel. When cancer presses on the spinal cord, Cancer Research UK calls it an emergency that needs treating quickly. Call your team right away, at any hour, or go to the emergency department.

How do doctors find out it is this?

• An MRI scan of the brain and the whole spine. The spine is checked because this tumor can spread through the fluid. Checking does not mean it has.
• The operation. Surgery to take the tumor out is usually where the piece of tumor comes from. It is tested for gene changes, because the gene result is part of the name.
• A spinal tap. A small needle goes into the lower back to take out a little of the fluid. The lab looks for tumor cells in it. A young child may be given medicine to sleep through it.

When the spinal tap happens varies. Some teams do it before the operation if they can. After surgery, fluid taken too soon can be muddied by the operation, so the sources say to wait. They give different waits, from about 10 days to about 3 weeks. Ask your team when yours will be.

What do the words on my report mean?

The rules for naming brain tumors changed in 2021. If your paperwork was written before that, some of its words are no longer used.

This matters more than it sounds. People read an old report next to a new one, see two different names, and think somebody made a mistake. Usually nobody did. The tumor did not change. The way doctors write it down changed.

Three changes affect almost every report.

• Roman numeral grades, such as grade II or grade IV on an older report. These are now written with ordinary numbers instead: grade 2, grade 4. Nothing about the grade itself changed. Only the way of writing it did.
• Gene results are now part of the name. Older reports named a tumor mostly by how the cells looked. A name written now usually has a gene result built into it. That is why a newer report can look longer and more technical than an older one about the same tumor. You may also see NOSNOS: Short for "not otherwise specified". It means the gene tests needed to name the tumor fully were not done or could not be done. Ask your team whether they can be. See the glossary or NECNEC: Short for "not elsewhere classified". The tests were done, but the results do not match any named tumor type. Ask your team what it means for your plan. See the glossary written next to your diagnosis. These say something about the testing, not about how serious the tumor is. NOS means the tests needed to be more exact were not available. NEC means testing was done, but the results did not fit any defined group neatly.

Seeing an older name on your own report does not mean it was wrong. It means it was written under the old rules. If you are holding an old report and a new one and they do not match, that is a good thing to take to your team and ask about.

The name on your report usually says where the tumor is, and then a gene result if one was found.

• Posterior fossa ependymoma, group PFA or group PFB. Low at the back of the brain. Group PFA is found mostly in babies and young children. Group PFB is found mostly in teenagers and adults.
• Supratentorial ependymoma, ZFTA fusion-positive. Higher up in the brain. Fusion-positive means a gene called ZFTA has joined onto another gene.
• Supratentorial ependymoma, YAP1 fusion-positive. Higher up in the brain, with a different gene joined. It is rare, and found mostly in babies.
• Spinal ependymoma, or spinal ependymoma, MYCN-amplified, which names a gene result in a tumor in the spinal cord.
• Myxopapillary ependymoma. Low in the spine. Grade 2.
• Subependymoma. Grade 1.
• Posterior fossa ependymoma or supratentorial ependymoma, with no group or gene after it. The name was given by place alone. That happens when the gene test was not done, did not work, or found something else.

A name may also have grade 2 or grade 3 beside it. For the names built on a gene result, a grade is often left off. There is not yet enough evidence to set one.

Your pathology report walks through the document itself.

If your older paperwork says something different

• Anaplastic ependymoma. An older name for a faster-growing ependymoma. It is no longer used. A report written now usually has grade 3 beside the name instead.
• Classic ependymoma. Reports used to split classic from anaplastic. That split is no longer used.
• RELA fusion-positive, or C11orf95-RELA. Older names for the ZFTA kind above.
• Myxopapillary ependymoma, grade 1. It is grade 2 now. The tumor did not change. It was moved because it comes back about as often as other ependymomas in the spine.
• Papillary, clear cell or tanycytic ependymoma. These now describe how the cells look. They are no longer separate kinds.
• PF-EPN-A, PF-EPN-B or ST-EPN-RELA. Research ways of writing the same names, and they turn up on gene test reports. PF-EPN-A is group PFA, PF-EPN-B is group PFB, and ST-EPN-RELA is the ZFTA kind. SE in one of these names, such as PF-EPN-SE, means subependymoma.

How is it usually treated?

A tumor board is a meeting where specialists look at one person's case together: surgeons, cancer doctors, the lab, and the people who read scans. It tends to happen when a case is complicated or something is hard to read.

You do not go to the meeting. Afterwards, the doctor who knows you best explains what was said.

What they decide is advice, not an order. The choice is still yours. Because you are not in the room, tell your team what matters to you before it happens, so it is taken into account.

Surgery is the main treatment. The surgeon aims to take all of it out, as long as that can be done without harming the parts of the brain or spine around it. How much came out explains the words the team will use afterwards. After surgery at the back of the brain, some children have posterior fossa syndromeposterior fossa syndrome: Something that can happen after an operation on the lower back of the brain. Some children cannot speak for a while. Swallowing, walking and mood can change too. It always starts in the first week after surgery. See the glossary, which the next section explains.

A second operation is sometimes offered, if the scan after surgery shows tumor left behind and taking the rest out looks possible. It may come after a short course of chemotherapy.

Radiation usually follows surgery for children and teens, aimed at the place where the tumor was. The American Cancer Society recommends it for most, to try to stop the tumor coming back, even when all of the tumor seems to be out. If tumor cells are found in the fluid, radiation may cover the whole brain and spine.

• Babies and young children. Radiation can harm a growing brain, so sources draw an age line, and they draw it in different places. St. Jude and a 2025 review say radiation is used from about age 1. That review adds that some children aged 1 to 3 have chemotherapy first, to put radiation off. The American Cancer Society's general page says radiation is usually not given before age 3. Ask your team where they draw the line, and why.
• Not every tumor gets radiation. Doctors are not sure it helps after a grade 2 tumor is fully taken out. A child whose grade 2 tumor higher in the brain was all removed may be watched with scans instead. For a tumor in the spinal cord, radiation is usually given only if some was left behind.
• Proton therapy is used more and more for children, to spare the healthy brain around the tumor. It is not available everywhere.

Chemotherapy has a smaller part. A trial in children and young adults, whose tumor was all or nearly all gone before radiation, found that adding chemotherapy after radiation did not help, at least so far. It is still used to put off radiation in young children, or to shrink a tumor before a second operation. If it is offered, it is fair to ask what it is for.

If the fluid is blocked, a shunt or another small operation can let it drain. Some shunts are only needed for a while.

Ask about clinical trials for your kind of ependymoma, and what testing they need.

What is treatment actually like, and what is normal afterwards?

The library pages carry the detail: what an operation is like, what radiation is like and proton therapy.

After surgery at the back of the brain

Some children get this after surgery for an ependymoma at the back of the brain.

A day or two after surgery low at the back of the brain, some children stop talking. It can also change swallowing, walking, mood and behavior. It always starts within the first week.

Suddenly not being able to speak is on the ambulance list above, and that rule still stands. What this section describes usually starts in the hospital, in the days right after the operation, where the team is watching for it.

It is frightening to see. Doctors know it as a complication of the operation itself. Most children slowly get better. Speech usually comes back over days or weeks, and for some it takes months. Most children walk on their own again. Some trouble with speech, movement or mood can last longer, even years. Speech therapy can help. If the swallowing, walking, mood or behavior changes start or get worse after you are home, call your team the same day, or right away if there is a shunt.

Before surgery, it is fair to ask how often your surgeon operates in this part of the brain. St. Jude says the risk of it is lower with an experienced surgeon.

After surgery on the spine

Pain, numbness and tiredness can go on for a while after spinal surgery. Before you go home, ask your team which of those to expect, and which mean calling them. New or worse pain in the back or neck, and weakness or numbness that is new or worse, is a right-away call, under the spinal cord rule above.

Everyday life: school, work, seizures and tiredness

• Living with seizures covers the practical side, and what to do during a seizure is worth reading before you need it.
• School and learning. Radiation to a young brain can affect memory and learning later on. Hearing and hormones can be affected too. Doctors check growth and development for years afterwards. Ask early who at the hospital helps with school.
• Driving rules depend on where you live. Ask your team directly.
• Tiredness can come from surgery and radiation, and it can also come from the tumor or from fluid that is not draining. Sleeping much more than being awake is a same-day call, or a right-away call if there is a shunt.

Ask early who at the hospital helps with work and money.

Follow-up scans, and what to do while you wait

The scans go on for years after this tumor. It can come back without causing any symptoms, and sometimes long after treatment ended. One recent review suggests scans for about 7 to 10 years. Another says nobody is yet sure how often or for how long. Scans are often every few months at first, and further apart later. Your team sets yours.

Keep going to the scans even after years of clear ones.

Follow-up scans goes through what each scan is compared with, and waiting for results covers the gap before the answer.

If it comes back, or changes

It can come back in the place it started, or somewhere else in the brain or spine. Both of those happen.

There are still choices. Your team may talk about surgery again, radiation again, chemotherapy, or a clinical trial.

Radiation a second time is something sources describe differently. Recent reviews say it can be an option for adults and for children. The CERN Foundation says someone who has had radiation before may not be able to have more. Ask your team whether it is an option for you, and what it would be for.

Some people get a second opinion at this point. The CERN Foundation says it can bring up other treatments and trials.

No choice here is the wrong one. Ask what each option is for, and write the answers down.

Did I cause this?

Almost everybody asks this, usually in the first week, usually in private. It is worth answering plainly.

For most brain tumors, nobody knows the cause. That is the honest state of it. The exact cause of most brain and spinal cord tumors is not fully understood, and there are very few well established risk factors. Tumors start when genes that control how cells grow and divide are changed, and in most cases those changes happen by chance during a person's life. Not because of something done or not done.

Cell phones. They give off a kind of radiation called radiofrequency. It is not the same as the radiation in an x-ray. The energy is too low to damage DNA, and it is not known to cause cancer. The evidence so far is that using a cell phone does not cause brain tumors.

Stress. There is no scientific evidence that stress causes brain tumors, and no data linking stress to a higher chance of getting one. A hard year before a diagnosis is a hard year. It is not a cause.

Something you ate, or did not eat. No food and no diet is an established cause of a brain tumor. It is not on the list of known risk factors at all.

Something you passed on, or caught. Brain tumors are not contagious. Most are not inherited either. A small number of people do have an inherited condition that raises the risk, and if that applies to you, your team will raise it and offer to talk it through.

If you are turning this over at three in the morning, that is ordinary and it happens to nearly everyone. It is also worth saying out loud to somebody on your team, because they have answered it many times and they would rather you asked.

Is it passed down in families? Usually not. Some ependymomas in the spinal cord happen in people with an inherited condition called NF2-related schwannomatosis. Older letters call it neurofibromatosis type 2, and the new name was suggested in 2022. If a relative has that condition, it is worth asking your team whether anyone else should be checked.

What might happen over time

For the person caring for someone with this

If you are the one driving to the appointments, keeping track of the pills, and lying awake at night worrying about them, this part is for you.

You are allowed to ask questions. In one study, families said they were afraid of annoying the doctor by asking too much. One of them was told by a doctor to stop playing doctor. Ask anyway. Write your questions down before you go, and bring a notepad. Two people in the room hear more than one.

Ask who your first call is. Care comes from a lot of people at once, and it is easy to end up as the only one holding it together. Ask the team: who do I call first? Some teams have one person whose job this is, often a nurse. Get the name and the number.

Get two numbers, and know which one to use. One for work hours, one for nights and weekends. Then ask what counts as "call us today" and what counts as "call an ambulance" for this tumor, and write the answer down where you can see it. If you are not sure which it is, call. Nobody on that team will mind.

If they have seizures, read about them before you need to. What to do when someone has a seizure is the one to know by heart. Living with seizures is about the rest of the time: what is safe, what to change, and driving.

Ask to be shown anything you are sent home to do. In that same study, families were sent home to change dressings and give medicines with no training at all. That is not fair to you. Before you leave the hospital, ask someone to show you. Then do it once while they watch.

Say what you notice. You may see a change before they do. Changes in thinking, mood and behavior can show up to the people close by first. Telling the team is not going behind their back. It is something the team cannot learn any other way.

Look after yourself, and let people help. Eat, sleep, and keep your own appointments. Take a little of the day back for yourself. When someone asks what they can do, give them a real job: a meal, a ride, an hour with the kids. Most people want to help and do not know how to offer.

You can talk to somebody about all of this today. See where to get help.

Three things specific to this diagnosis.

Many people caring for someone with an ependymoma are parents of a young child. Everything above still applies, and it is fine to ask the team to explain things more than once.

After surgery at the back of the brain, watch talking and swallowing. If your child comes home still having trouble swallowing, ask to be shown how to give food and drink before you leave. New or worse trouble swallowing after you are home is a same-day call, or a right-away call if there is a shunt. Choking, or trouble breathing, is an ambulance call.

Keep the scan dates somewhere safe. The scans go on for years, and you may be the one keeping track of them.

What to ask your team

• Where exactly is the tumor, and what is its full name, with any gene result?
• Was all of it taken out? What did the scan after surgery show?
• Would a second operation help?
• Has the whole spine been scanned, and has the fluid been checked?
• Is radiation recommended, and why? What would happen if we waited?
• Could proton therapy be an option?
• If chemotherapy is offered, what is it for?
• Before surgery at the back of the brain: how often do you do this operation?
• How long will the scans go on, and how often?
• Is there a clinical trial for this kind?
• What would make you want to see us before the next appointment?
• Who do we call after hours, and what number is it?

Where to get support

• Getting help now if you need somebody today.
• The CERN Foundation, a program of the National Brain Tumor Society, is set up for people affected by ependymoma. It has a guide for people who are newly diagnosed, and it connects people with others who have been through it.
• Together by St. Jude has pages for families of children and teens, including one on posterior fossa syndrome.
• What to do during a seizure, worth reading before you need it.
• Living with seizures for the practical side.
• Your team's specialist nurse, who is usually the fastest way to get a question answered.