Ependymoma in Children

Overview

Ependymoma is a rare type of brain cancer, mostly seen in children. The average age at diagnosis is 5 years. Approximately 200 to 300 cases are diagnosed annually in the United States. Nobody knows the cause of these tumors.

Ependymomas develop from cells lining the ventricles, located deep within the brain. These ventricles are normally filled with cerebrospinal fluid (CSF), and help cushion the brain from trauma.

Signs & symptoms

Ependymomas can arise in both the cerebral hemispheres — the large part of the brain — and in the cerebellum.

In the cerebral hemispheres, they can cause seizures, or loss of function leading to weakness or numbness in parts of the body. In the cerebellum, ependymomas often interfere with the flow of CSF within the brain, causing a build-up of CSF known as hydrocephalus. Hydrocephalus is often associated with headaches, or a rapidly increasing head circumference in infants, and is often the first symptom of ependymoma.

Diagnosis

As with other brain tumors, imaging studies such as a CT scan or MRI can define the size and spread of a tumor and may suggest the diagnosis of ependymoma. However, a surgical biopsy or removal is required to confirm the diagnosis.

Treatment

Ependymoma is a serious condition that needs to be treated by a team consisting of neurosurgeons, neuro-oncologists and radiation oncologists. Even with appropriate therapies, a fraction of patients cannot be cured.

As with most tumors, treatments are limited to three modalities: surgery, radiation and chemotherapy. Surgery is the most effective therapy for ependymomas, and is used whenever possible. Because of their deep location within the brain, however, it is often difficult to completely remove ependymomas without damaging vital brain structures.

Depending on the tumor's location, your child's neurosurgeon may remove as much of the tumor as possible by creating an opening in the skull in an operation called a craniotomy. If the ependymoma can't be removed due to its location, surgery may be limited to a biopsy or partial removal of the tumor.

The prognosis for children with ependymomas varies widely depending on the characteristics of the tumor and other factors, including location and percentage of tumor removed by surgery. The percentage of tumor removed by the surgeon is the most important factor in determining prognosis. Patients whose tumors can be completely removed have a much better prognosis than patients with incompletely removed ependymomas.

Although there is controversy regarding additional treatment for ependymoma, radiation therapy is often used when a tumor cannot be entirely removed. Chemotherapy is generally ineffective for ependymoma.