Ependymoma

What Is an Ependymoma?

Ependymomas are cancerous tumors that grow in your brain or any part of the spine, including your neck and upper and lower back. They form at first in your ependymal cells in the middle of your spinal cord and in the fluid-filled spaces in your brain known as ventricles.

Unlike other kinds of cancer, ependymomas usually don’t spread to other parts of your body. But they can spread to more than one area of your brain or spine. In children, these tumors are likely to come back after treatment.

If you learn that you or your child has an ependymoma, there's a lot to take in. You'll have lots of questions about how to treat the disease and manage the swirl of emotions you may feel.

Depending on where the cancer is, your doctor may recommend treatment with surgery, chemotherapy, or radiation. But make sure you take care of your mental health, too. Turn to family, friends, and support groups to get the emotional backing you need.

Ependymoma Symptoms

Most ependymomas start out small and grow slowly over years, so you may not notice any problems at first.

When symptoms start, the way you feel depends on where the tumor is. 

If the tumor is in your brain:

  • You may have seizures or frequent headaches
  • You may also feel nauseous or throw up. 
  • You could lose your balance. 
  • You could get blurry vision.
  • You may feel confused or irritable.

If it’s in your spine, you may have: 

  • Stiffness or pain in your neck or back.
  • Weakness or numbness in your legs or arms
  • A hard time peeing
  • Sexual problems

If your baby has an ependymoma, one of the first symptoms you may notice is that they have a larger than usual head.

Ependymoma Causes

Doctors aren't sure what causes ependymomas. But your chances of getting the disease go up if you have a genetic condition called neurofibromatosis type 2.

Ependymomas affected men and women equally. Among adults, you're most likely to get the tumors between your 40s and your 60s. For children, about 30% of ependymoma cases happen in kids under 3.

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Types of Ependymomas

You may hear your doctor talk about which type of ependymoma you have. The doctor will usually tell you the grade first and then the subtype within that grade. There are four kinds, based on where your cancer starts and how fast it grows.

Grade I (subependymomas). These usually show up near a ventricle in the brain and grow slowly. They usually affect adults and older men.

Grade I (myxopapillary ependymomas). These usually appear in young adults’ spinal cords. They grow slowly.

Grade II (ependymomas). The most common type of ependymoma, this tumor is usually found in your brain.

Grade III (anaplastic ependymomas). These almost always happen in your brain or skull and typically grow faster than other types of ependymomas.

Most ependymomas in young kids grow near the base of the brain. The majority of spinal tumors affect those over age 12.

Ependymoma Diagnosis

The doctor may do these tests to see if you have an ependymoma:

  • Physical exam . The doctor checks your body and asks about your health and medical history.
  • Neurological exam. The doctor will have you move around to look at how your brain, nerves, and spinal cord are working.
  • MRI (magnetic resonance imaging). This scan makes detailed pictures of your organs. You'll likely get a shot of a dye that collects around cancer cells so they show up better in pictures.
  • Spinal tap. You'll lie down on your side and the doctor will use a needle to collect fluid from your spine. Lab technicians will analyze it for signs of tumors.
  • Biopsy. The only way to be sure whether a tumor is an ependymoma is to put a small piece under a microscope to look for cancer cells. This is done during surgery. It also lets your doctor know the type and grade of the tumor.  

Ependymoma Treatments

You'll work with a team of doctors, including an oncologist, neurosurgeon, and neurologist, to decide on your  treatment. They’ll consider many things, including you or your child’s age, the location of the tumor and the grade, and possible side effects. Options include:

  • Observation. Because many ependymoma tumors grow slowly, if you don't have symptoms you may not get treated. Instead, the doctor may watch your symptoms and follow up with yearly MRIs.
  • Surgery. If your tumor is larger or causes symptoms, the doctor may suggest surgery with small instruments. For spinal tumors, the surgeon will make a small cut into your spinal cord to remove the tumor. For brain tumors, the surgeon removes part of your skull and cuts out a tiny piece of brain tissue. They'll check the cells under a microscope. If they find cancer cells, they’ll take out as much of the tumor as possible during the same surgery. Most of the time they can remove the tumors safely and you won't have side effects from the operation. You might feel slightly less sensation in your legs after spinal cord surgery. After surgery, you’ll get an MRI to check that the surgeon removed the entire tumor. If part of it is still in your body, you may need a second operation to take out the rest.
  • Radiation or chemotherapy. Even if your surgeon removes all of the tumor, they'll likely suggest chemotherapy or radiation to kill any remaining cancer cells. Radiation is riskier for children under 3 because it raises the chances of growth and developmental problems. Talk to your doctor about the best option for your young child.
  • Targeted drug therapy. Certain drugs are being tested that attack cancer cells. These may do less harm to nearby healthy cells than other kinds of treatment.

As you or your child goes through treatment, reach out to those close to you. Their support and guidance can help you manage the emotional rollercoaster you may find yourself on. Your doctor can also suggest a counselor or therapist you can talk to.

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Ependymoma Outlook

You or your child may have long-lasting side effects related to treatment for the tumor. It can take weeks or months for them to show up. Problems may be physical, like headaches and fatigue, or emotional, like depression. Children may also have learning or growth delays.   

Most people treated for ependymoma live for 5 years or more. The rate is slightly lower for children under age 19.

It’s not unusual for an ependymoma to grow back after treatment, especially in children. If it does come back, it’s usually in the same spot as the original tumor. Your child will need regular checkups to make sure they stay cancer free. 

WebMD Medical Reference Reviewed by Hansa D. Bhargava, MD on July 29, 2020

Sources

SOURCES:

American Brain Tumor Association: "Ependymoma."

American Cancer Society: "Types of Brain and Spinal Cord Tumors in Adults," "Types of Brain and Spinal Cord Tumors in Kids."

Collaborative Ependymoma Research Network: "Ependymoma Overview," "Fast Facts" FAQs About Ependymomas,” “Treatment,” “Risks and Complications,” “Ependymoma Statistics,” “Recurrence.” 

Columbia University Department of Neurological Surgery: "Ependymoma."

Dana Farber-Boston Children's Cancer and Blood Disorders Center: "Ependymoma."

Medscape: "Ependymoma."

National Cancer Institute: "Childhood Ependymoma Treatment," "Definition: Cerebrospinal Fluid," "Definition: Ependymal Cell," "Definition: Ventricle," "What You Need to Know About Brain Tumors," Ependymoma.”

St. Jude Children's Research Hospital: "Ependymoma."

UpToDate: "Neurofibromatosis Type 2."

American Society of Clinical Oncology: “Ependymoma - Childhood Guide.”

Mayo Clinic: “Brain Tumor: Ependymoma.”

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