Ependymoma

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.

Symptoms

Ependymoma signs and symptoms may depend on where it happens. This tumor can form in the brain or spinal cord.

Symptoms of ependymoma in the brain include:

• Blurry vision.
• Confusion.
• Headaches.
• Irritability.
• Nausea.
• Seizures.
• Vomiting.

Symptoms of ependymoma in the spinal cord include:

• Bowel changes.
• Loss of balance.
• Pain in the neck or back.
• Trouble walking.
• Weakness in the legs.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

Causes

It's not clear what causes ependymoma. This tumor starts as a growth of cells in the brain or spinal cord.

Ependymoma happens when cells in the brain or spinal cord develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In tumor cells, the DNA changes give different instructions. The changes tell the tumor cells to grow and multiply quickly. Tumor cells can keep living when healthy cells would die. This causes too many cells.

Risk factors

Factors that increase the risk of ependymoma include:

• Young age. Ependymoma can happen at any age. This cancer happens most often in children.
• Inherited syndromes. Some ependymomas are linked to genetic conditions passed down in families, such as with neurofibromatosis type 2 and Li-Fraumeni syndrome.

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.

Preparing for an appointment

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

If your healthcare professional thinks you might have ependymoma, you may be referred to a doctor who specializes in diseases of the nervous system, called a neurologist. If a cancer diagnosis is made, you also may be referred to a doctor who specializes in treating cancer, called an oncologist.

Because appointments can be brief, it's a good idea to be prepared. Here's some information to help you get ready.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins or supplements you're taking and the doses.
• Take a family member or friend along. Sometimes it can be very hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For ependymoma, some basic questions to ask include:

• Do I have an ependymoma?
• Is my ependymoma cancerous?
• What is the grade of my ependymoma?
• Has my ependymoma spread to other parts of my body?
• Will I need more tests?
• What are the treatment options?
• How much does each treatment increase my chances of a cure or prolong my life?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• Is there one treatment option you believe is the best?
• What would you recommend to a friend or family member in my situation?
• Should I see a specialist?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?
• What will determine whether I should plan for a follow-up visit?

Don't hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your symptoms begin?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?