All About Pediatric Ependymoma

What is ependymoma?

An ependymoma is a central nervous system tumor that is most often found in the brain or spinal cord. This cancer starts in the ependymal cells. Ependymal cells line the ventricles in the brain and the central canal in the spinal cord. They help make cerebrospinal fluid (CSF).

What causes ependymoma and who is at risk?

Each year about 1,098 people are diagnosed with ependymoma. It can affect both adults and children. In children, the tumor is more common in the lower half of the brain and in adults, it is more common in the spine. More males are diagnosed than females and it is also more common in white and non-Hispanic people.

The cause of ependymoma is not known. Children with neurofibromatosis type 2 (NF2) are at an increased risk of ependymoma because NF2 increases the risk of central nervous system tumors.

How can ependymoma be prevented in a child?

There is no way to prevent ependymoma because there are no known risk factors besides NF2.

What screening tests are used?

There are no recommended screening tests for ependymoma. A child won't be tested unless they are showing signs or symptoms of the disease.

What are the signs and symptoms?

The symptoms of ependymoma vary from child to child. They can be caused by pressure on a certain part of the brain from the tumor. Some symptoms include:

• Headache.
• Changes in vision such as blurriness or quick, jerky eye movements.
• Nausea and vomiting with or without a headache.
• New trouble walking or keeping balance.
• Feeling more tired than usual.
• Neck pain.
• Not growing as fast as expected or not reaching developmental milestones as expected.
• Seizures.

Each child can have different symptoms. If your child is having any changes in their normal activity, growth, or is having any changes in health, they should be seen by their provider.

How is childhood ependymoma diagnosed?

A number of tests can be used to help diagnose ependymoma:

• To start, your child will have a full physical examination. The provider will ask you and your child about symptoms.
• A neurological exam, which includes questions and tests, will be done. This checks how well the brain, spinal cord, and nerves are working.
• Imaging tests such as an MRI may also be done to see what is going on inside the brain and body.
• A lumbar puncture (LP) may be done to remove and test the cerebrospinal fluid. The fluid will be placed under a microscope to look for cancer cells.
• Depending on the results of all of these tests, a biopsy may be done. A biopsy is the use of surgery or a procedure to remove part of or all of the tumor. A pathologist will look at the piece of the tumor under a microscope to make a diagnosis. In cases of ependymoma, a biopsy may be used to both diagnose and treat the cancer. Part of the skull will be removed and tools will be used to remove as much of the tumor as possible.

How is ependymoma staged?

Staging is often used in cancer to help create a treatment plan. Staging takes into account how large a tumor is, how it looks under a microscope and if it has spread. There is no standard staging system for ependymoma.

The World Health Organization groups these tumors into 5 subtypes:

• Subependymoma.
• Myxopapillary ependymoma.
• Ependymoma.
• RELA fusion-positive ependymoma.
• Anaplastic ependymoma.

The tumor may also be given a grade. This describes how abnormal the cancer cells look under a microscope. The World Health Organization has 3 grades for ependymoma:

• Grade I: Subependymoma.
• Grade II: Ependymoma or myxopapillary ependymoma.
• Grade III: Anaplastic ependymoma.

The classification and grade information will help guide a treatment plan.

How is childhood ependymoma treated?

It is important to find a pediatric facility that has experience treating patients with ependymoma. A team of specialists, along with you and your child, will determine the best treatment plan. A major factor when determining the treatment plan is whether the whole tumor can be removed with surgery. Something to consider when planning a treatment course is that there is potential for late or long-term side effects, meaning side effects that can occur after treatment has ended. Late effects of treatment for ependymoma can include issues with hearing, thyroid problems, neurological issues, fertility problems, issues with growth and development, and in rare cases a second cancer, amongst others. The provider should speak about these possible side effects.

Surgery

Surgery to remove the tumor is often the treatment of choice. In some cases a tumor can only be partially removed or not removed due to size or its location in the brain. In those cases other treatments will be used. Surgery to remove the tumor is called a craniotomy. Part of the skull is removed and tools are used to remove the tumor.

Radiation

Radiation is the use of high-energy x-rays to kill cancer cells. It can be used to treat ependymoma that can't be removed by surgery. If the cancer has spread (metastasized) it can be used to treat the whole brain and spine. It can also be used after surgery.

Chemotherapy

Chemotherapy is the use of medications that kill cancer cells. It can be given into a vein (IV), into a muscle, by mouth, into the cerebrospinal fluid, or into the tumor itself. Chemotherapy given by itself without other treatments has not been found to effectively treat ependymoma. Chemotherapy may be used between surgeries to help shrink the tumor and make it easier to remove. Studies are being done to see if treatment with radiation and chemotherapy after surgery is beneficial.

Clinical Trials

There are clinical research trials for most types of cancer, and every stage of the disease. Clinical trials are designed to determine the value of specific treatments. Trials are often designed to treat a certain stage of cancer, either as the first form of treatment offered, or as an option for treatment after other treatments have failed to work. They can be used to evaluate medications or treatments to prevent cancer, detect it earlier, or help manage side effects. Clinical trials are extremely important in furthering our knowledge of disease. It is through clinical trials that we know what we do today, and many exciting new therapies are currently being tested. Talk to your provider about participating in clinical trials in your area. You can also explore currently open clinical trials using the OncoLink Clinical Trials Matching Service.

Follow-up Care and Survivorship

After treatment for childhood cancer, you will be followed closely to monitor for the cancer coming back, to manage ongoing side effects, and to transition to survivorship. At first you will have frequent appointments with providers and have ongoing tests to monitor your health, and possible recurrence of your cancer. As time goes on, these visits and testing will become less frequent. The oncology team will discuss the plan for follow up.

What can you do to live a healthy life after treatment? There is no supplement or specific food you can eat to assure good health, but there are things you can do to live healthier, prevent other diseases, detect cancers early and work with the social and emotional issues, including insurance, employment, relationships, sexual functioning, and fertility, that a prior cancer diagnosis sometimes brings with it. Your oncology team is there to support you and can help you find support resources.

It is important to have a plan for who will provide follow up care (an oncologist, survivorship doctor or primary care doctor). Talk with the team about developing a survivorship care plan or develop one using the Smart ALACC tool. Your child may benefit from being seen in a survivorship clinic. At a survivorship clinic a provider can review your child's history and provide recommendations. You can contact cancer centers in your area to see if they have a survivor's clinic or search for a clinic on OncoLink's survivorship clinic list.

Resources for More Information

American Brain Tumor Association

Pediatric Brain Tumor Foundation