Ependymomas are tumors in the central nervous system, which consists of the brain and spinal cord, and can develop during childhood. These tumors develop from cells that line the spaces within the brain (ventricles) or spinal cord. Ependymomas are usually cancerous (malignant).
Symptoms depend on the location of the tumor and may include headaches, vomiting, listlessness, and problems with balance.
Diagnosis is based on an imaging test of the brain and a biopsy.
Treatment includes surgery, radiation therapy, and sometimes chemotherapy.
The prognosis depends on how much of the cancer can be removed and on genetic features of the tumor.
Ependymomas are the third most common central nervous system tumor in children and account for 5 to 10% of central nervous system tumors in children. (The central nervous system is made up of the brain and spinal cord.) Most children diagnosed with an ependymoma in the brain are about 5 to 8 years old, whereas most children with an ependymoma in the spine are about 12 years old. Ependymomas can occur in infants.
Starting from the top right label:
Astrocytoma (glioma)
Optic nerve glioma
Craniopharyngioma
Pituitary adenoma
Schwann cell tumor (nerve sheath tumor)
Ependymoma (glioma)
Brain stem glioma
Medulloblastoma
Pineal gland tumor
Meningioma
Oligodendroglioma (glioma)
designua/stock.adobe.com
In children, most ependymomas develop in or near the area in the back of the brain at the bottom of the skull called the posterior fossa. This area contains the cerebellum (which helps control coordination and balance) and the brain stem (which controls critical body functions such as breathing).
Ependymomas also occur in the upper part of the brain called the supratentorial area. This area contains the cerebrum, fluid-filled spaces (ventricles), choroid plexus, hypothalamus, pineal gland, pituitary gland, and optic nerve.
Sometimes ependymomas develop in the spinal cord.
Symptoms of Ependymomas in Children
Symptoms depend on the location of the tumor.
For ependymomas in the supratentorial area, symptoms can include changes in mood or personality, difficulty concentrating, headaches, seizures, and abnormal function of a specific area of the body.
For ependymomas in the posterior fossa, symptoms are typically related to increased pressure within the skull (intracranial pressure). Infants may not meet developmental milestones. They may be irritable, have an increased head circumference, and have no appetite. Older children have nausea, vomiting, headaches, listlessness, and problems with balance, coordination, and walking.
For ependymomas in the spinal cord, symptoms may include back pain, weakness, numbness/tingling, and difficulty controlling urination and bowel movements.
Diagnosis of Ependymomas in Children
Magnetic resonance imaging (MRI) and biopsy
Genetic testing of the tumor
The diagnosis of ependymoma is based on results of an MRI scan.
If a tumor is found, a sample is taken and sent to a laboratory for examination and a genetic analysis.
Treatment of Ependymomas in Children
Surgery and radiation therapy
Sometimes chemotherapy
(See also Cancer Treatment Principles.)
Doctors first surgically remove as much of the tumor as is safely possible.
Radiation therapy increases the rate of survival and is usually done after surgery.
Chemotherapy does not seem to increase the rate of survival, but, in some children, it may help shrink the tumor before surgery.
Prognosis for Ependymomas in Children
How well the child does depends on how much of the tumor can be removed. Children who have all or almost all of the tumor removed have a better chance of survival than those whose tumor can be only partly removed.
Children who survive are at risk of developing problems with their brain, spinal cord, and nerves because of the side effects of surgery and radiation therapy.
More Information
The following English-language resource may be useful. Please note that The Manual is not responsible for the content of this resource.
American Cancer Society: How to Cope if Your Child Has Cancer
