Ependymoma

Date Updated: 11/19/2021

Overview

Ependymoma is a type of tumor that can form in the brain or spinal cord. Ependymoma begins in the ependymal cells in the brain and spinal cord that line the passageways where the fluid (cerebrospinal fluid) that nourishes your brain flows.

Ependymoma can occur at any age, but most often occurs in young children. Children with ependymoma may experience headaches and seizures. Ependymoma that occurs in adults is more likely to form in the spinal cord and may cause weakness in the part of the body controlled by the nerves that are affected by the tumor.

Surgery is the primary treatment for ependymoma. For more aggressive tumors or for tumors that can't be removed completely with surgery, additional treatments, such as radiation therapy or chemotherapy, may be recommended.

Diagnosis

Tests and procedures used to diagnose ependymoma include:

Neurological exam. During a neurological exam, your doctor will ask you about your child's signs and symptoms. He or she may check your child's vision, hearing, balance, coordination, strength and reflexes. Problems in one or more of these areas may provide clues about the part of your child's brain that could be affected by a brain tumor.
Imaging tests. Imaging tests can help doctors determine the location and size of the brain tumor. MRI is often used to diagnose brain tumors, and it may be used along with specialized MRI imaging, such as magnetic resonance angiography. Because ependymoma can occur in both the brain and spine, imaging tests should be used to create pictures of both areas when a diagnosis of ependymoma is suspected.
Removing cerebrospinal fluid for testing (lumbar puncture). Also called a spinal tap, this procedure involves inserting a needle between two bones in the lower spine to draw out fluid from around the spinal cord. The fluid is tested to look for tumor cells or other abnormalities.

Based on your child's test results, the doctor may suspect ependymoma and recommend surgery to remove the tumor. Once removed, the tumor cells will be tested in a laboratory to confirm the diagnosis. Specialized tests are used to determine the types of cells and their level of aggressiveness, which the doctor may use to guide treatment decisions.

Treatment

Ependymoma treatment options include:

Surgery to remove the ependymoma. Brain surgeons (neurosurgeons) work to remove as much of the ependymoma as possible. The goal is to remove the entire tumor, but sometimes the ependymoma is located near sensitive brain or spinal tissue that makes that too risky.

If the entire tumor is removed during surgery, your child may not require any additional treatment. If some tumor remains, the neurosurgeon may recommend another operation to try to remove the rest of the tumor. Additional treatments, such as radiation therapy, may be recommended for more aggressive tumors or if all of the tumor can't be removed.
Radiation therapy. Radiation therapy uses high-energy beams, such as X-rays or protons, to kill cancer cells. During radiation therapy, your child lies on a table while a machine moves around him or her, directing beams to precise points in the brain.

Radiation therapy may be recommended after surgery to help prevent more aggressive tumors from recurring or if neurosurgeons weren't able to remove the tumor completely.

Specialized techniques can help to ensure that treatment delivers radiation to the tumor cells and spares as much of the surrounding healthy tissue as possible. Conformal radiation therapy, intensity-modulated radiation therapy and proton therapy are types of radiation therapy that allow doctors to carefully and precisely deliver radiation.
Radiosurgery. Technically a type of radiation and not an operation, stereotactic radiosurgery focuses multiple beams of radiation on precise points to kill the tumor cells. Radiosurgery is sometimes used when an ependymoma recurs after surgery and radiation.
Chemotherapy. Chemotherapy uses drugs to kill cancer cells. Chemotherapy isn't very effective for most cases of ependymoma. The role of chemotherapy is still largely experimental and is reserved for certain situations, such as when the tumor grows back despite surgery and radiation.
Clinical trials. Clinical trials are studies of new treatments. These studies give you a chance to try the latest treatment options, but the risk of side effects may not be known. Ask your doctor whether your child might be eligible to participate in a clinical trial.