The central nervous system is made up of the brain and spinal cord. Ependymomas are slow-growing central nervous system tumors that develop from cells lining the spaces within the brain (ventricles) or spinal cord. Ependymomas are usually cancerous (malignant).
The cause of ependymomas is not known.
Symptoms depend on the location of the tumor and may include headaches, vomiting, listlessness, and problems with balance.
Diagnosis is made by an imaging test and a biopsy.
Treatment includes surgery, radiation therapy, and sometimes chemotherapy.
The prognosis depends on the child's age and on how much of the cancer is removed.
Ependymomas are the third most common central nervous system tumor in children, accounting for 10%. The majority of children diagnosed with ependymoma are about 6 years of age. However, about one third of cases occur in children younger than 3 years of age.
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
Symptoms of ependymomas 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
Magnetic resonance imaging (MRI) and biopsy
The diagnosis of ependymoma is based on results of an MRI.
If a tumor is found, a sample is taken and sent to a laboratory for examination (biopsy).
Treatment of Ependymomas
Surgery, radiation therapy, and sometimes chemotherapy
(See also Cancer Treatment Principles.)
Initial therapy is surgical removal of 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 or radiation therapy.
Prognosis for Ependymomas
How well the child does depends on the child's age, type of ependymoma, and on how much of the tumor can be 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: If Your Child Is Diagnosed With Cancer: A resource for parents and loved ones of a child who has cancer that provides information about how to cope with some of the problems and questions that come up just after a child is diagnosed
