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Childhood Craniopharyngioma Treatment (PDQ®)

Last modified: 2014-05-22
Last downloaded: 2014-09-15

   

General Information About Childhood Craniopharyngioma

   

Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland.

Childhood craniopharyngiomas are rare tumors usually found near the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves).

Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain.

Craniopharyngiomas are usually part solid mass and part fluid-filled cyst. They are benign (not cancer) and do not spread to distant parts of the brain or to other parts of the body. However, they may grow and press on parts of the brain or other nearby areas, including the pituitary gland, the optic chiasm, optic nerves, and fluid-filled spaces in the brain. Many functions including hormone making, growth, vision, and normal working of the brain may be affected. Benign brain tumors need treatment.

This summary discusses the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary. See the PDQ treatment summary on Childhood Brain and Spinal Cord Tumors Treatment Overview for information about the different types of childhood brain and spinal cord tumors.

Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. (See the PDQ summary on Adult Brain Tumors for more information.)

  

There are no known risk factors for childhood craniopharyngioma.

Craniopharyngiomas are rare in children younger than 2 years of age and are most often diagnosed in children aged 5 to 14 years. It is not known what causes these tumors to form.

  

Signs of childhood craniopharyngioma include vision changes and slow growth.

These and other signs and symptoms may be caused by craniopharyngiomas or by other conditions. Check with your child’s doctor if your child has any of the following:

  • Headaches including morning headache or headache that goes away after vomiting.
  • Vision changes.
  • Nausea and vomiting.
  • Loss of balance or trouble walking.
  • Increase in thirst or urination.
  • Increase in head size (in infants).
  • Unusual sleepiness or change in energy level.
  • Unusual changes in personality or behavior.
  • Short stature, slow growth, or delayed puberty.
  • Hearing loss.
  

Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
  • Visual field exam: An exam to check a person’s field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). Any loss of vision may be a sign of a tumor that has damaged or pressed on the parts of the brain that affect eyesight.
  • MRI (magnetic resonance imaging) of the brain and spinal cord with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain. A substance called gadolinium is injected into a vein. The gadolinium collects around the tumor cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
  • Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. For example, the blood may be checked for unusual levels of thyroid-stimulating hormone (TSH) or adrenocorticotropic hormone (ACTH). TSH and ACTH are made by the pituitary gland in the brain.
  

Childhood craniopharyngiomas are diagnosed and may be removed in the same surgery.

Doctors may diagnose a craniopharyngioma based on where the tumor is in the brain and how it looks on a CT scan or MRI. Sometimes a biopsy is required to find out the type of tumor. The biopsy is done during surgery, and a tissue sample is removed, using one of the following procedures:

  • An open biopsy (a hollow needle is inserted through a hole in the skull into the brain).
  • A computer-guided needle biopsy (guided by a computer, a hollow needle is inserted through a small hole in the skull into the brain).
  • A transsphenoidal biopsy (instruments are inserted into part of the brain by going through the nose and the sphenoid bone, a butterfly-shaped bone at the base of the skull).

A pathologist views the tissue under a microscope to look for tumor cells. If tumor cells are found, the doctor may remove as much tumor as safely possible during the same surgery.

  

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The size of the tumor.
  • Where the tumor is in the brain.
  • Whether the tumor is solid or a fluid-filled cyst.
  • Whether there are tumor cells left after surgery.
  • The child's age.
  • Whether the tumor has just been diagnosed or has recurred (come back).

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Stages of Childhood Craniopharyngioma

  

The process used to find out if cancer has spread within the brain or to other parts of the body is called staging. There is no standard system for staging childhood craniopharyngioma. Craniopharyngioma is described as newly diagnosed disease or recurrent disease.

The results of the tests and procedures done to diagnose craniopharyngioma are used to help make decisions about treatment.


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Recurrent Childhood Craniopharyngioma

Recurrent craniopharyngioma is a tumor that has recurred (come back) after it has been treated. The tumor may come back in the same area of the brain where it was first found.


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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 and symptoms that begin before diagnosis and continue for months or years.

Signs and 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 and 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. An endoscope and a curette are inserted through the nose and sphenoid sinus to remove cancer from the pituitary gland.

Transsphenoidal surgery. An endoscope and a curette are inserted through the nose and sphenoid sinus to remove cancer from the pituitary gland.

Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.

Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.

  • 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. 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.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.


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Treatment Options for Childhood Craniopharyngioma

Newly Diagnosed Childhood Craniopharyngioma

Treatment of newly diagnosed childhood craniopharyngioma may include the following:

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.

Recurrent Childhood Craniopharyngioma

Craniopharyngioma may recur (come back) no matter how it was treated the first time. Treatment options for recurrent childhood craniopharyngioma are based mainly on what type of treatment was given before and the needs of the individual child. Treatment options may include:

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.


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To Learn More About Childhood Craniopharyngioma and Other Childhood Brain Tumors

For more information about childhood craniopharyngioma and other childhood brain tumors, see the following:

For more childhood cancer information and other general cancer resources, see the following:


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Changes to This Summary (05/22/2014)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.


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Back to Top Source: The National Cancer Institute's Physician Data Query (PDQ®) Cancer Information Summaries (http://www.cancer.gov/cancertopics/pdq)