What Is Childhood Ependymoma?

Childhood ependymoma is a rare type of cancer that forms in a child's brain or spinal cord. It starts in cells that line the ventricles (fluid-filled spaces in the brain) as well as in the canal that holds the spinal cord. Nearly half of ependymomas are diagnosed in kids under age 3.

Your doctor has many options to treat ependymoma. Some of these treatments are already in use. Others are being tested in clinical trials. To give your child the best possible outcome, you’ll need to see a team of doctors who specialize in this type of cancer.

What Causes It?

Doctors don't know why some children get ependymoma.

Children with neurofibromatosis type 2 (NF2) may be at higher risk for it. NF2 is an inherited disease that causes tumors to form in the nervous system.

What Are the Symptoms?

It depends on where the tumor is located. The most common ones are:

How Is It Diagnosed?

Doctors called pediatric oncologists treat children who have cancer. To start, the doctor will ask about your child's symptoms and health history.

He’ll also ask questions and do some tests to see how well your child's brain and spinal cord are working. This is called a neurological exam. Your doctor will check your child's reflexes, senses, and thinking ability.

Other tests used to diagnose ependymoma include:

  • MRI. This test uses a powerful magnet and radio waves to make pictures of your child's brain and spinal cord. It can show the location and size of a tumor. Your child might get medicine to help him sleep so he stays still during the test. Before the MRI, he might get an injection of gadolinium into a vein. This substance makes the cancer show up clearer on the picture.
  • Computed tomography, or CT. This test uses X-rays and a computer to make detailed pictures inside the body. Your child might have dye injected into a vein before the test to help the doctor see the tumor more easily.
  • Lumbar puncture (spinal tap). In this test, the doctor places a needle into your child's back and removes a small sample of spinal fluid. Medicine is used to numb the area first. A lab technician checks the fluid under a microscope to see if it contains cancer cells.
  • Biopsy. This is the only test that can confirm your child has ependymoma. The surgeon removes a small piece of brain tissue through a needle. If cancer cells are present, the doctor will remove the tumor during the same surgery.

After the tests, your doctor will give the cancer a grade based on how fast it is likely to grow. Low-grade tumors grow more slowly than high-grade ones. Once your doctor knows the cancer grade, he can better plan your child's treatment.

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What's the Treatment?

It depends on your child’s age, where the cancer is located, and whether it has spread. Ependymoma often grows very slowly. If the cancer is small and hasn’t spread, your doctor might suggest observation. This means the doctor will closely watch your child's cancer but won't treat it right away.

For a larger or faster-growing ependymoma, the main treatments are:

Surgery

The procedure used to treat ependymoma is called a craniotomy. The doctor makes a small opening in your child's skull and takes out as much of the tumor as possible.

Often, it's hard to take out the whole tumor without damaging important structures around it.

Some children will need another MRI after the surgery to see if any part of the tumor is still there. If so, a second surgery will be done.

Your child might get chemotherapy or radiation after surgery. These treatments kill off any cancer cells that are left behind.

Radiation Therapy

This treatment delivers high-energy X-rays to the tumor to kill cancer cells or stop them from growing. Most of the time ependymoma is treated with external radiation therapy. That means the radiation comes from a machine outside your child's body. Doctors use radiation after surgery. They might also use it when they can't perform surgery for some reason.

Side effects from radiation include:

Most of these symptoms will go away once the treatment is finished. In children under age 3, radiation could affect growth and development. Newer types of radiation like proton-beam therapy might reduce this risk.

Chemotherapy

This treatment uses strong medicine to kill cancer cells or stop their growth. Your child might get these drugs by mouth, or by insertion into a vein.

Sometimes, doctors combine two or more chemotherapy drugs to treat childhood ependymoma. Or, your child might get both chemotherapy and radiation to help these treatments work better.

Side effects of chemotherapy include:

  • Fatigue
  • Nausea and vomiting
  • Appetite loss
  • Hair loss
  • Diarrhea
  • Increased risk for infection

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What Happens After Treatment?

The outlook for kids who are treated for ependymoma is good, especially if doctors can remove the whole tumor. Afterward, your child will need careful follow-up with the doctor to make sure the cancer doesn't come back. He'll also need to be checked for side effects from the cancer treatments.

WebMD Medical Reference Reviewed by Dan Brennan, MD on February 02, 2019

Sources

SOURCES:

American Society for Clinical Oncology: "Ependymoma -- Childhood – Diagnosis," "Ependymoma -- Childhood -- Risk Factors," "Ependymoma -- Childhood -- Treatment Options."

Macmillan Cancer Support: "Ependymomas."

National Cancer Institute: "Childhood Ependymoma Treatment (PDQ) -- Patient Version."

Penn Medicine: "All About Pediatric Ependymoma."

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

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