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Ependymoma is a tumor of the brain and spinal cord. It is the 3rd most common malignant brain tumor in children. There are about 200 new cases of pediatric ependymoma in the United States each year.
Ependymomas are most common in children under 5 years of age. However, they may develop at any age.
Ependymomas grow from cells that line the fluid-filled ventricles of the brain and central canal of the spinal cord.
These tumors can sometimes spread through the cerebrospinal fluid (CSF).
In children, about 75% of ependymomas occur in the posterior fossa region of the brain, but they can also develop in other areas of the central nervous system (CNS). Depending on the location of the tumor, different parts of the CNS may be affected including the:
Many ependymomas form in the fourth ventricle in the posterior fossa. These tumors are most likely to affect the brain stem and cerebellum.
Treatment for ependymoma includes surgery to remove as much of the tumor as possible. Radiation therapy is often used along with surgery to kill any remaining cancer cells. Chemotherapy may also be used before or after surgery.
The survival rate for childhood ependymoma is 50-70%. Prognosis depends on the location of the tumor, the amount of tumor that can be removed with surgery, and features of the tumor cells. Doctors are learning more about the biology and molecular features of ependymoma. This information can help predict how a tumor might respond to treatment.
Ependymoma can come back after treatment, and children often need long-term care to watch for recurrence.
Most ependymomas occur in infants and young children under 5 years old.
Certain changes in genes and chromosomes within the tumor cell are associated with ependymoma. Usually, it is not known why these genetic changes occur.
Some children may be at increased risk for brain tumors including ependymoma due to neurofibromatosis type 2 (NF2), a rare inherited condition.
Signs and symptoms of childhood ependymoma depend on several factors including the size and location of the tumor and the child’s age and stage of development.
Ependymoma symptoms may include:
As the tumor grows, it often blocks the normal flow of cerebrospinal fluid. This causes a buildup of fluid within the brain known as hydrocephalus. The fluid increases pressure in the brain (intracranial pressure). Many of the symptoms of ependymoma are due to hydrocephalus.
Ependymoma tumors are identified by how they look under the microscope. They are generally classified as grade I, grade II, or grade III tumors. The more abnormal the cells look, the higher the grade. Children with ependymoma usually have tumors that are grade II (classic ependymoma) or III (anaplastic ependymoma).
Certain types of ependymoma tend to have a better outcome than other types. However, other factors also affect treatment and prognosis.
|Tumor subtypes based on histology
|Grade II||Classic ependymoma (includes papillary, clear cell, and tanycytic ependymoma)|
|Grade III|| Anaplastic ependymoma
The overall 10-year survival for pediatric ependymoma is about 50-70%. The prognosis depends on multiple factors. Children with certain types of ependymoma tend to have a better chance for long-term survival. However, late recurrences or relapse (beyond 5 years) may occur.
Factors that influence chance of cure include:
Ependymoma is hard to cure without successful surgery to remove the tumor. When surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor.
Treatment of ependymoma usually includes surgery followed by focal radiation therapy. Some patients may also receive chemotherapy. Treatment depends on the type and location of tumor, age of the child, and whether the tumor is newly diagnosed or recurrent.
Some children with hydrocephalus due to ependymoma may have a shunt placed in the brain to prevent cerebrospinal fluid from building up. A shunt is a small tube that drains fluid from the brain.
Current risk-based approaches to ependymoma treatment classify patients based on specific tumor features and predicted outcomes. Doctors consider:
The goal of a risk-based approach is to achieve cure while lowering the risk of treatment side effects. For low risk patients, lower intensity therapy can reduce long-term problems from radiation and chemotherapy. For high-risk patients, more intensive therapy can improve chance of survival.
Incorporating 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.
Ongoing follow-up care, laboratory tests, and routine imaging are needed to monitor patients for recurrence of disease and other medical problems. The care team will set a schedule based on type of tumor, response to treatment, and individual patient needs.
Later relapse of ependymoma is more common compared to some other cancers. Most patients who relapse do so within 5 years of their diagnosis. However, these tumors can also recur after the 5-year milestone of cancer survival.
Survivors of CNS tumors are at risk for problems such as decreased cognitive function, hearing loss, sleep problems, hormone problems, stroke, and second cancers. These problems may be caused by the tumor itself or develop as 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 therapy.
Reviewed: July 2019