What is Childhood Craniopharyngioma?

Craniopharyngioma is a rare type of brain tumor that mostly affects children between the ages of 5 and 14. Adults sometimes get them, too. They grow near the pituitary gland at the base of the brain. Craniopharyngiomas are made up of solid parts and fluid-filled pockets called cysts.

This type of tumor isn't cancer, so it can't spread to other parts of your body. But it can get larger and press on parts of your brain that control vision and hormone release.

Doctors aren’t sure why these tumors form, and there’s no known way to prevent them.

Most craniopharyngiomas grow slowly and are treatable. To make sure your child has the best outcome, you should try to work with doctors who have experience in treating this kind of tumor.

What Are the Symptoms?

As the tumor grows, it can put pressure on the pituitary gland -- which produces hormones -- and the optic nerve. This sends signals from the eyes to the brain. The pressure causes many symptoms, which include:

How Is It Diagnosed?

To start, the doctor will ask about your symptoms and health history. Tests of your nervous system health -- called a neurologic exam -- will evaluate your coordination, reflexes, senses, and thinking ability.

An eye doctor will examine your visual field. That means how well you can see straight ahead of you and to each side. This test can show whether you've lost vision, which could be a sign that a tumor is pressing on parts of your brain that control sight.

Other tests your doctor might use to diagnose this tumor include:

  • MRI. It uses powerful magnets and radio waves to make pictures inside your brain. First, you'll get a substance called gadolinium injected into a vein. This makes the tumor show up clearer on the picture.
  • CT, or computed tomography. It's a powerful X-ray that makes detailed pictures inside your body. Dye might be injected into a vein before the test to make the tumor show up better on the picture.
  • Blood tests. These check for levels of hormones and other substances in your blood. A higher-than-normal hormone level can be a sign that a tumor is pressing on your pituitary gland.

The only way to be sure you have a craniopharyngioma is with a biopsy. The doctor will insert a needle or other tools into your skull and remove a small piece of tissue. A specialist in a lab will look at the tissue sample under a microscope to see if it contains any tumor cells.

Continued

What's the Treatment?

The main one is surgery to remove as much of the tumor as possible. The surgery can be done in a couple of ways:

  • Transsphenoidal surgery. The surgeon makes a cut below the nose or upper gum. Then the instruments are placed through the opening into the brain.
  • Craniotomy. The surgeon makes an opening in the skull and removes the tumor. Or if part of the tumor is a fluid-filled cyst, the doctor might place a tube called a catheter into the tumor to drain it.

Sometimes, surgery can cause side effects. They include bleeding and damage to parts of the brain that control hormone release, vision, arm and leg movements, and other functions.

Radiation therapy is another treatment for craniopharyngioma. It uses high-energy X-rays to kill cancer cells or stop them from growing. You might get radiation after surgery to destroy any tumor cells that were left behind.

Radiation is delivered in one of two ways:

  • External radiation therapy. Radiation is delivered through a machine outside your body.
  • Internal radiation therapy. This method places needles, seeds, wires, or other substances with radiation on them next to the tumor inside your brain.

Side effects of radiation include:

In children, radiation can cause long-term learning and growth problems. Your doctor might wait to use this treatment, adjust the dose, or use a different kind of radiation to reduce the risks. Proton radiotherapy is a newer form of radiation that uses protons instead of X-rays. It may cause fewer side effects in children.

Life After Treatment

The outlook for people with craniopharyngioma is good, because surgery and other treatments can usually remove the whole tumor. But this tumor and its treatments can affect the body's hormone production. After treatment, you or your child might need to see an endocrinologist -- a doctor who treats hormone problems.

Your child will have follow-up visits with her doctor for many years after her treatment. The doctor will help her manage any side effects from the tumor or its treatment, and check to see if the tumor has come back.

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

Sources

SOURCES:

American Brain Tumor Association: "Craniopharyngioma."

American Society of Clinical Oncology: "Craniopharyngioma -- Childhood: Treatment Options."

Cancer Research UK: "Treatment for Craniopharyngioma."

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

Penn Medicine: "All About Craniopharyngioma."

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

© 2019 WebMD, LLC. All rights reserved.

Pagination