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Ependymoma is a tumor of the brain and spinal cord. About 200 children are diagnosed with ependymoma in the United States each year.
Ependymomas are most common in children under age 5. But these tumors may happen at any age. Rarely, they can spread through the cerebrospinal fluid (CSF). This fluid surrounds and protects the brain and spinal cord.
In children, about 75% of ependymomas are in the posterior fossa region of the brain. This space near the base of the skull contains the cerebellum and brain stem. But ependymomas can also form in other areas of the central nervous system (CNS).
The parts of the CNS that are affected depend on the tumor’s location. These include the:
Many ependymomas form in the 4th ventricle in the posterior fossa. These tumors are most likely to affect the brain stem and cerebellum.
Treatment for ependymoma includes:
The survival rate for childhood ependymoma is 50–70%. Prognosis depends on:
Doctors are learning more about the biology and molecular features of ependymoma. These details can help predict how a tumor might respond to treatment. Ependymoma has 10 molecular subtypes with different clinical features.
Ependymoma can come back after treatment. Children often need long-term care to watch for this.
Signs and symptoms of childhood ependymoma depend on several factors. These include the tumor's size and location and the child’s age and stage of development.
Ependymoma symptoms may include:
As the tumor grows, it may block the normal flow of CSF. This causes a fluid buildup in the brain known as hydrocephalus. The fluid increases pressure in the brain. Many symptoms of ependymoma are due to hydrocephalus.
Most ependymomas occur in infants and children under age 5.
Certain changes in genes and chromosomes in the tumor cells are linked to ependymoma. These are not usually passed down from parents. In most cases, it is not known why these genetic changes occur.
Some children may have a higher risk for ependymoma due to neurofibromatosis type 2 (NF2). This rare condition is passed through families.
Doctors use these tests to diagnose ependymoma:
Treatment of ependymoma usually includes surgery followed by radiation therapy. Some patients may also get chemotherapy.
Treatment depends on:
Risk-based approaches to treatment classify patients based on tumor features and predicted outcomes. Doctors consider:
Some children with hydrocephalus due to ependymoma may have external drains or shunts placed in the brain to prevent CSF from building up.
The overall 10-year survival rate for pediatric ependymoma is about 50–70% in the United States.
Factors that affect the chance of cure include:
Children with certain types of ependymoma tend to have a better chance for long-term survival. But late recurrences or relapse (beyond 5 years) may occur.
Ependymoma is hard to cure without successful surgery to remove the tumor. If surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor.
Prognosis for ependymoma depends on many factors. Every case is different. Always talk to your care team about your child’s case.
Palliative care and support services such as rehabilitation, psychology, and social work can help ependymoma patients and families manage symptoms, promote quality of life, and make care decisions. Talk to your care team about what problems to expect and ways to help manage them.
Patients need ongoing follow-up care, lab tests, and routine imaging to watch for disease recurrence and other medical problems. The care team will set a schedule based on the tumor type, response to treatment, and individual patient needs.
General follow-up schedule for ependymoma survivors:
Survivors are at risk for problems such as:
These problems may be caused by the tumor or they may be long-term or late effects related to treatment. Regular checkups and screenings by a primary care physician are important to watch for health problems that can develop years after treatment ends.
Reviewed: July 2023