Ependymoma in children — a clear, honest guide for parents

Ependymoma is a type of brain or spinal cord tumour that arises from ependymal cells — the cells that line the fluid-filled spaces (ventricles) inside the brain and the central canal of the spinal cord. In children, ependymoma most commonly develops inside the brain, particularly in the posterior fossa (the lower back part of the brain near the brainstem and cerebellum). In most cases, no single clear cause is identified. Ependymoma is not caused by anything the family did or did not do. Research has identified certain molecular changes within the tumour cells that influence how the tumour behaves and how it should be treated, which is why genetic and molecular testing of the biopsy sample has become a routine part of diagnosis at specialist centres.

The symptoms of ependymoma depend on where the tumour is located and how much pressure it is placing on surrounding brain or spinal cord tissue. Tumours in the posterior fossa — the most common location in children — often cause symptoms related to raised pressure inside the skull, because they can block the normal circulation of cerebrospinal fluid. Common warning signs include: persistent morning headaches that may wake a child from sleep; repeated vomiting, especially in the morning and without an obvious cause; clumsiness, balance problems, or a change in the way the child walks; neck stiffness or the child holding their head at an unusual angle; blurred or double vision; and an unusual squint. In younger children who cannot describe symptoms, parents may notice a bulging fontanelle (soft spot on the head), unusual irritability, or a slowing of developmental milestones. Spinal ependymoma may cause back or neck pain, weakness in the legs or arms, or changes in bladder and bowel control. These symptoms alone do not confirm ependymoma — they each have many possible causes. When they persist or worsen over days to weeks, a doctor's evaluation is important.

Diagnosis begins with imaging. An MRI of the brain and spine is the standard first investigation — it identifies the tumour's location, size, and whether any cells appear to have spread through the cerebrospinal fluid to other parts of the central nervous system. CT scanning may also be used in urgent situations. However, imaging alone cannot confirm the diagnosis or tell doctors how the tumour is likely to behave. Definitive diagnosis requires surgery, during which a neurosurgeon removes as much of the tumour as is safely possible (a procedure called maximal safe resection) and sends the tissue to a pathologist. The pathologist examines the tissue under a microscope, classifies the tumour type and grade, and runs additional molecular tests to identify the tumour's specific biological subtype. These molecular results are now central to deciding the best treatment approach, so they should be done at a centre with paediatric neuro-oncology expertise.

Ependymoma is a serious diagnosis, but it is treatable, and many children do well after treatment — particularly when the tumour is detected before it has spread and when surgery achieves a thorough removal. The extent of surgical removal (how completely the tumour is taken out) is one of the most important factors that influences long-term outcomes. After surgery, radiation therapy is usually recommended for children who are old enough to receive it safely, because it reduces the risk of the tumour returning. The treatment plan for each child is decided by a multidisciplinary team that includes a paediatric neurosurgeon, a radiation oncologist, a paediatric medical oncologist, and a neuropathologist — the tumour is reviewed at a team meeting before any recommendation is made. At CION, every paediatric case goes through our full tumor board before a treatment protocol is recommended. We walk this journey with you and your child, one step at a time.

After surgery, the child's MRI is repeated — usually within 24 to 72 hours — to assess how much tumour remains. The pathology report from the removed tissue, along with the molecular subtype results, guides the next decision. In most children over three years of age who have a localised tumour, focal radiation therapy to the tumour site is recommended after surgery. The radiation plan is carefully designed to treat the tumour bed while protecting as much healthy brain tissue as possible. Whether chemotherapy is used depends on the child's age, the tumour grade, whether there is any residual tumour after surgery, and the molecular subtype. Younger children (typically under three) may receive chemotherapy first, to delay or reduce radiation to the developing brain. Regular MRI scans during and after treatment monitor for any signs of regrowth. The CION team will explain the specific schedule and what to watch for at home.

Treatment for childhood ependymoma can be demanding, and some children experience effects that persist after treatment ends. Radiation to the brain, particularly in younger children, can affect learning, memory, and concentration in the years that follow — this is why the radiation plan is designed to protect healthy brain tissue, and why younger children may receive chemotherapy first to delay radiation. Surgery near the cerebellum or brainstem may temporarily affect balance, speech, or swallowing — a period called cerebellar mutism syndrome in some children, which usually improves over weeks to months with rehabilitation support. Hormone-related effects can occur if the radiation field is near the pituitary gland. The CION team includes coordination with rehabilitation specialists, speech therapists, and educational support services, because healing extends beyond medicine. Follow-up care continues for years after treatment to monitor both tumour control and the child's development.