What is an ependymoma?
An ependymoma is a tumor that arises from the ependymal cells, which line the brain and spinal cord. Over 90 percent of ependymoma originate from the brain and 10 percent from the spinal cord. Ependymomas rarely spread (metastasize) from their site of origin.
Ependymomas are classified as either supratentorial (in the cerebral hemispheres) or infratentorial (in the back of the brain). Variations of this tumor type include subependymoma, subependymal giant-cell astrocytoma, and malignant ependymoma.
Ependymomas are the third most common type of central nervous system (CNS) tumor in children (following astrocytoma and medulloblastoma). Approximately 200 cases of childhood ependymoma are diagnosed in the U.S. each year. Ependymomas account for between six percent and 12 percent of brain tumors in children less than 18 years of age, but 30 percent of brain tumors in children less than three years of age. The average age at diagnosis is four to six years of age.
What are the symptoms of an ependymoma?
Symptoms of ependymoma are determined by where they are located within the brain or spine.
Symptoms of ependymoma in the brain typically include headache, weakness, visual field cut or seizures, double vision. Ependymoma of the brain often cause hydrocephalus (too much cerebrospinal fluid) that can lead to headache, vomiting, poor balance, and decreased level of consciousness.
Symptoms of spinal cord ependymoma may include weakness and/or loss of sensation, pain, and bowel or bladder dysfunction, and possibly severe pain in the lower back and legs.
How is an ependymoma diagnosed?
Patients suspected of having a brain or spinal cord tumor undergo a magnetic resonance imaging (MRI) scan of the brain and spine to further define the location of the tumor and to assess if there is any metastasis (spread) of the tumor. A biopsy of the tumor is required to make the final diagnosis of an ependymoma and to subtype the ependymoma according to World Health Organization guidelines. Patients with ependymoma of the brain require a spinal tap (lumbar puncture) to assess for any spread of the tumor through the spinal fluid.
How is an ependymoma treated?
The initial treatment for ependymoma is surgery. In general, the neurosurgeon will attempt to remove as much of the tumor as possible, without causing damage to the normal brain. A complete resection, confirmed by post-operative MRI or computed tomography (CT) scan, often yields a favorable prognosis. Although total resection is optimal, it is not always possible because vital structures can be involved by the tumor.
Children whose have tumor spread within the cerebrospinal fluid (metastatic disease) benefit from craniospinal radiation therapy.
Chemotherapy is reserved for patients with residual tumor following surgery (incomplete surgical resection) with the goal to shrink the tumor and make it more amenable to a second surgery. Chemotherapy is also used in children younger than three years of age in an attempt to delay radiation therapy until they are older.
About treatment for ependymoma at Children’s Hospitals and Clinics of Minnesota
Our cancer and blood disorders program consistently achieves excellent results ranking it in the top ten programs in the United States. Children’s Hospitals and Clinics of Minnesota treats the majority of children with cancer and blood disorders in Minnesota and provides patients access to a variety of clinical trials using ground-breaking new treatments. Through our renowned program, patients experience unparalleled family support, a nationally recognized pain management team, and compassionate, coordinated care.
If you are a family member looking for a Children’s neuro-oncologist, please call our clinic at (612) 813-5940.
If you are a health professional looking for consultation or referral information, please call Children's Physician Access at 1-866-755-2121 (toll-free) and ask for the on-call hematologist/oncologist.
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