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Childhood Craniopharyngioma Treatment (PDQ®)

Patient Version

Treatment Option Overview

There are different types of treatment for children with craniopharyngioma.

Different types of treatments are available for children with craniopharyngioma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with tumors. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because tumors in children are rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and health care team.

Children with craniopharyngioma should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with tumors. The pediatric oncologist works with other pediatric healthcare providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:

Childhood brain tumors may cause signs or symptoms that begin before diagnosis and continue for months or years.

Signs or symptoms caused by the tumor may begin before diagnosis and continue for months or years. It is important to talk with your child's doctors about signs or symptoms caused by the tumor that may continue after treatment.

Some treatments for tumors cause side effects months or years after treatment has ended.

Side effects from tumor treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of tumor treatment may include the following:

  • Physical problems such as vision problems and obesity.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).

For example, serious late effects of radiation therapy for craniopharyngioma may include the following:

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects tumor treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).

Five types of treatment are used:

Surgery

The way the surgery is done depends on the size of the tumor and where it is in the brain. It also depends on whether the tumor has grown into nearby tissue in a finger-like way. The types of surgery include the following:

  • Transsphenoidal surgery: A type of surgery in which the instruments are inserted into part of the brain by going through an incision (cut) made under the upper lip or at the bottom of the nose between the nostrils and then through the sphenoid bone (a butterfly-shaped bone at the base of the skull).
    Transsphenoidal surgery; drawing shows an endoscope and a curette inserted through the nose and sphenoid sinus to remove cancer from the pituitary gland. The sphenoid bone is also shown.
    Transsphenoidal surgery. An endoscope and a curette are inserted through the nose and sphenoid sinus to remove cancer from the pituitary gland.
  • Craniotomy: Surgery to remove the tumor through an opening made in the skull.
    Drawing of a craniotomy showing a section of the scalp that has been pulled back to remove a piece of the skull; the dura covering the brain has been opened to expose the brain. The layer of muscle under the scalp is also shown.
    Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.

Sometimes all of the tumor that can be seen is removed in surgery and no further treatment is needed. At other times, it is more difficult to remove the tumor because it is growing into or pressing on nearby organs. Serious problems may occur if the pituitary gland, hypothalamus, optic nerves, or carotid artery are affected during surgery.

If there is tumor remaining after the surgery, radiation therapy is usually given to kill any tumor cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

After surgery, follow-up testing with MRI will be done for several years to check if the tumor has come back. Also, if part of the pituitary gland is removed in surgery, several types of pituitary hormone replacement pills will be given for life.

Surgery with cyst drainage

Surgery may be done to drain tumors that are mostly cyst. This lowers pressure in the brain and relieves symptoms. A catheter (thin tube) is inserted into the cyst and a small container is placed under the skin. The fluid drains into the container and is later removed. Sometimes, after the cyst is drained, a drug that causes the inside wall of the cyst to scar is put through the catheter into the cyst. This is done to stop the cyst from making fluid or to increase the amount of the time it takes for the fluid to build up again. Surgery to remove the tumor may be done after the cyst is drained.

Limited surgery and radiation therapy

Limited surgery is used to treat some craniopharyngiomas. It is used to diagnose the tumor, remove fluid from a cyst, and relieve any pressure on the optic nerves. Limited surgery is followed by radiation therapy. This treatment option may have fewer serious side effects than surgery to remove tumor from the pituitary gland or hypothalamus.

Radiation therapy is a tumor treatment that uses high-energy x-rays or other types of radiation to kill tumor cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the tumor. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the tumor. The way the radiation therapy is given depends on the type of tumor being treated and whether it has spread.

Because radiation therapy to the brain can affect growth and development in young children, ways of giving radiation therapy that have fewer side effects are being studied. These include:

  • Stereotactic radiosurgery: For very small craniopharyngiomas at the base of the skull, stereotactic radiosurgery may be used. Stereotactic radiosurgery uses a head frame to aim radiation at the tumor only. This treatment is also called stereotaxic radiosurgery.
  • Intracavitary radiation therapy: Intracavitary radiation therapy is a type of internal radiation therapy that may be used in tumors that are part solid mass and part fluid-filled cyst. Radioactive material is sent directly into the tumor, and causes less damage to the nearby hypothalamus and optic nerves.
  • Intensity-modulated proton therapy: A type of radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. A computer is used to target the exact shape and location of the tumor with proton therapy. This type of 3-dimensional radiation therapy may cause less damage to healthy tissue in the brain and other parts of the body. Proton radiation is different from x-ray radiation.

Chemotherapy

Chemotherapy is a treatment that uses anticancer drugs to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach tumor cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid or an organ, the drugs mainly affect tumor cells in those areas (regional chemotherapy). Intracavitary chemotherapy is a type of regional chemotherapy that places drugs directly into a cavity, such as a cyst. The way the chemotherapy is given depends on the type of tumor being treated.

Biologic therapy

Biologic therapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. For craniopharyngioma, the biologic therapy drug is placed directly inside the tumor using a catheter.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood craniopharyngioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.

Patients can enter clinical trials before, during, or after starting their treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients who have not improved. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the disease or decide how to treat it may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed. These tests are sometimes called follow-up tests or check-ups.

  • Updated: October 9, 2014