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Overview

Ependymoma is a growth of cells that forms in the brain or spinal cord. The cells form a mass called a tumor. Ependymoma begins in the ependymal cells. These cells line the passageways that carry cerebrospinal fluid. This fluid surrounds and protects the brain and spinal cord.

There are different types of ependymomas. Some grow slowly and aren't considered cancerous. Noncancerous tumors also are called benign tumors. A benign ependymoma may grow to press on nearby tissue. Sometimes ependymomas grow more quickly and are considered cancerous. Cancerous tumors also are called malignant tumors. A malignant ependymoma may grow to invade and destroy nearby tissue. Cancerous cells can break away and spread within the brain or spinal cord.

Ependymoma can occur at any age, but most often happens 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. It may cause weakness in the part of the body controlled by the nerves affected by the tumor.

Surgery is the primary treatment for ependymoma. For tumors that are growing quickly or for tumors that can't be removed completely with surgery, additional treatments may be recommended. These may include radiation therapy, radiosurgery, chemotherapy or targeted therapy.

Diagnosis

Tests and procedures used to diagnose ependymoma include:

• Neurological exam. During a neurological exam, a healthcare professional checks vision, hearing, balance, coordination, strength and reflexes. Problems in one or more of these areas may provide clues about the part of the brain or spinal cord that could be affected by a tumor.
• Imaging tests. Imaging tests make pictures of the body. They can show the location and size of an ependymoma. MRI is often used to diagnose brain tumors. It may be used along with specialized MRI imaging, such as magnetic resonance angiography. Because ependymoma can happen in the brain and the spinal cord, imaging tests may be used to create pictures of both areas.
• Cerebrospinal fluid testing. Called a lumbar puncture or spinal tap, this procedure involves inserting a needle between two bones in the lower spine. The needle draws out fluid from around the spinal cord. The fluid is tested to look for tumor cells.

Based on the test results, your healthcare professional may suspect ependymoma and recommend surgery to remove the tumor. Once removed, the tumor cells are tested in a lab to confirm the diagnosis. Special tests can tell the healthcare team about the types of cells involved in the tumor. Your healthcare team may use this information to guide treatment decisions.

Treatment

Ependymoma treatment options include:

Surgery

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

If the entire tumor is removed during surgery, additional treatment may not be needed. 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 cancerous tumors or if all of the tumor can't be removed.

Radiation therapy

Radiation therapy uses powerful energy beams to kill tumor cells. The energy can come from X-rays, protons or other sources. During radiation therapy, a machine directs beams of energy to specific points to kill the tumor cells there.

Radiation therapy may be recommended after surgery to help prevent cancerous tumors from coming back. It also may be recommended if neurosurgeons weren't able to remove the tumor completely.

Some special types of radiation therapy help focus the radiation treatment on the tumor cells. These special types of radiation may reduce the risk of damage to nearby healthy cells. Examples include conformal radiation therapy, intensity-modulated radiation therapy and proton therapy.

Radiosurgery

Stereotactic radiosurgery is an intense form of radiation treatment. It aims beams of radiation from many angles at the tumor. Each beam isn't very powerful. But the point where the beams meet gets a very large dose of radiation that kills the tumor cells.

Chemotherapy

Chemotherapy uses medicines to kill tumor cells. Chemotherapy isn't often used to treat ependymoma. It might be an option in certain situations, such as when the tumor grows back despite surgery and radiation.

Targeted therapy

Targeted therapy uses medicines that attack specific chemicals in the tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die. Targeted therapy might be an option to treat an ependymoma that comes back after treatment.

Clinical trials

Clinical trials are studies of new treatments. These studies offer a chance to try the latest treatment options, but the risk of side effects may not be known. Talk with your healthcare team if you're interested in a clinical trial.