Ependymoma: symptoms, treatment, types and outlook in adults and children

Macmillan Cancer Support says an ependymoma is a type of tumour of the brain and spinal cord that belongs to a group called gliomas. Gliomas are made up of cells that look like glial cells, the supporting cells in the brain and spinal cord. An ependymoma is made up of cells that look like ependymal cells, which line the fluid-filled spaces (ventricles) in the brain and the centre of the spinal cord. It can develop in any part of the brain or spine and at any age.

Cancer Research UK says that in adults, most ependymomas start in the ependymal cells that line the spinal cord, and that they can spread into the fluid that surrounds the brain.

Cancer Research UK says surgery is the main treatment, and you may also have radiotherapy and, less commonly, chemotherapy. Macmillan adds that your treatment depends on the size and position of the tumour, its type (including grade and molecular markers) and your symptoms. A team of specialists plans treatment, and you should have time to discuss choices and give consent.

Surgery: a neurosurgeon removes as much of the tumour as can safely be removed. Macmillan says some types of ependymoma that can be completely removed do not usually need any other treatment.

Radiotherapy uses high-energy X-rays to destroy tumour cells. Macmillan says you may have it if the tumour has not been completely removed, if it is a type more likely to come back, if you have a faster-growing ependymoma to reduce the risk of it coming back, or as your main treatment if surgery is not possible. Cancer Research UK says you usually have radiotherapy to the whole brain and spinal cord if the tumour has spread to the cerebrospinal fluid, or just to the area of the tumour if no tumour cells are found in the fluid.

Chemotherapy: Macmillan says it is rarely used for ependymoma in adults, though doctors may suggest it if the tumour comes back.

Macmillan says you may need treatment for symptoms before treatment for the tumour, and during and after your main treatment. Options include anti-epileptic drugs to reduce the risk of seizures, steroids to reduce swelling around the tumour, and surgery to drain fluid from the brain to another area of the body, for example an endoscopic third ventriculostomy (ETV) to relieve pressure inside the skull.

Cancer Research UK explains that some childhood tumours cause fluid to build up in the brain (hydrocephalus). Treatment is an operation, either a hole made to drain fluid into another area of the brain (ventriculostomy) or a tube called a shunt that drains fluid, usually to the tummy.

Macmillan says symptoms depend on the size and position of the tumour and whether it is in the spinal cord or the brain. Ependymomas are often slow growing and symptoms may develop slowly over many months.

• Spinal cord: pain in the neck or back, difficulty walking, numbness or weakness in the arms, and problems with bladder control (Macmillan).

• Brain: headaches, feeling or being sick, problems with coordination and balance, problems with sight, seizures (fits), confusion and problems with speech. Changes in mood and personality are rare (Macmillan).

• Cancer Research UK also lists dizziness and changes in eyesight for brain tumours, and neck pain for spinal tumours.

The Brain Tumour Charity says ependymoma is the third most common childhood brain tumour, with an average age at diagnosis of five years and at least a quarter of diagnoses before the age of 2. Most childhood ependymomas are found in the hindbrain (posterior fossa). Cancer Research UK says boys are affected more than girls, and around 30 children a year in the UK are diagnosed.

Symptoms can include a twisted or tilted head (torticollis), loss of balance, problems speaking, difficulty swallowing, clumsy movements, headaches, seizures, double vision, nausea and vomiting (The Brain Tumour Charity). Cancer Research UK adds irritability, lacking energy, not gaining weight as they should and, in babies, an increasing head circumference or a swollen soft spot. It notes that many symptoms are non-specific and similar to less serious childhood illnesses, and can be present for a few weeks or months before diagnosis.

Treatment is usually surgery first. The Brain Tumour Charity says that if a low-grade tumour can be completely removed, the child may not need any other treatment. Cancer Research UK says very young children are more likely to have side effects from radiotherapy, so they usually have chemotherapy instead after surgery. Proton beam therapy, a type of radiotherapy that uses protons and aims to cause less damage to healthy tissue around the tumour, can work well for some, but not all, children with ependymoma.

Macmillan says doctors usually use a brain CT scan first and an MRI scan of the brain, sometimes of both the brain and the spine. You may also have a biopsy, usually at the same time as surgery to remove the tumour, and a lumbar puncture to collect a sample of cerebrospinal fluid to test. Your doctor may examine your nervous system, check your eyes for swelling, ask questions to check reasoning and memory, and arrange blood tests.

Cancer Research UK says a specialist looks at a sample of the tumour under a microscope and may do molecular tests for specific genetic changes. These help identify the type and grade and which treatments may work best. For children, it says a lumbar puncture is usually done after surgery and that sedation or a general anaesthetic may be needed to help a child lie still.

Cancer Research UK says the information is based on the 2021 World Health Organisation classification. For ependymomas the grade does not always fit with how they behave, so it may not tell you much about how likely the tumour is to grow or spread. Macmillan notes that your tumour may not be given a grade because molecular tests may provide more information. It says only three grades are used for ependymomas: grades 1 and 2 are low grade and slow growing, and grade 3 is higher grade and faster growing.

Macmillan says the causes of ependymomas are unknown, but research is under way. In a small number of people it is linked to an inherited condition called neurofibromatosis type 2 (NF2). Cancer Research UK adds that in children there is a small increased risk with some inherited cancer syndromes, rarely including familial adenomatous polyposis (FAP) and spinal tumours in NF2, but not every child with one of these syndromes develops a brain tumour.

The Brain Tumour Charity stresses that there is nothing you or your child did, or could have avoided doing, that would have stopped an ependymoma from growing.

The Brain Tumour Charity says how long someone can live with an ependymoma is affected by many factors, including the grade, size and location of the tumour, the body's response to treatment and general health. The sources used here do not give a single survival figure, so ask your specialist team what applies to you.

If the tumour comes back, Cancer Research UK says your specialist might recommend more radiotherapy or chemotherapy to shrink it, and some people might have further surgery. Treatments can slow the growth of the tumour and help you feel better. Macmillan says if a tumour cannot be removed or stops responding, you can still have symptom treatment and supportive (palliative) care.

Cancer Research UK says that after treatment you have regular appointments and may have an MRI scan every 3 to 6 months for two years, with scans continuing after that depending on your situation. Speak to your specialist nurse if you have symptoms between appointments.

Macmillan says some side effects from treatment may continue, including tiredness or problems with processing thoughts and ideas, and some can start months or years later. You may be referred to a neurological rehabilitation service, which can arrange physiotherapy, speech and language therapy or occupational therapy. Cancer Research UK notes that a brain tumour can affect your ability to drive: ask your doctor or specialist nurse about your right to drive.

The sources used here do not set out emergency criteria. As general advice, see a GP promptly for persistent headaches, repeated vomiting, new problems with balance, vision or walking, new weakness or numbness, or bladder or bowel changes. Call 999 if someone has a seizure for the first time, loses consciousness, has sudden severe weakness, or is very drowsy or confused. If you are under a cancer team, use the specialist nurse contact you were given for any new symptom.

On the NHS, brain and spinal tumours are managed by a specialist multidisciplinary team in a neurosciences centre. Children are cared for in a children's cancer centre.

Some people seek a private neurosurgical or oncology opinion. Because ependymoma is rare, it is sensible to ask whether the team treats this tumour regularly and how the private team will link with your NHS multidisciplinary team, so that scans, molecular results and follow-up stay joined up.