Craniopharyngioma is a benign (noncancerous) central nervous system tumor (CNS) tumor. A tumor begins when cells start to grow uncontrollably, eventually forming a mass. There are many different types of central nervous system (CNS) tumors. Some are malignant (cancerous), which means they can invade other tissues and spread to other areas of the body. Others, such as craniopharyngioma, are less aggressive, called either low grade or benign, which means they are usually slow-growing and not as likely to spread.

The brain and spinal column make up the CNS, where all vital functions of the body are controlled. The brain is the center of thought, memory, and emotion. It controls the five senses (smell, touch, taste, hearing, and sight), movement, and other basic body functions, including heartbeat, circulation, and breathing. The spinal cord consists of nerves that carry information from the body to the brain and from the brain to the body. When a tumor begins in the CNS, it can affect a person’s thought processes and movements. A tumor in the CNS can also be difficult to treat because the tissue surrounding the tumor may be vital to the body’s functioning.

Craniopharyngioma starts near the pituitary gland in a part of the brain called the suprasellar region. This region is the area of the brain above the part of the skull where the pituitary gland is located. The pituitary gland is an important gland in the brain and is often called the “master endocrine gland” because it produces several different hormones that affect many bodily functions.

Craniopharyngioma is a slow-growing tumor that can grow for many years before being found. It can be solid and/or cystic (a closed pouch or sac that contains fluid), and it may contain calcified nodules (a growth or lump that has hardened due to deposits of calcium). A cystic tumor may also contain some solid material or debris and often contains very high amounts of protein.

Statistics

Approximately 200 new cases of craniopharyngioma are diagnosed each year in the United States. More than half occur in people younger than 18. Craniopharyngioma accounts for approximately 4% of childhood CNS tumors.

Cancer statistics should be interpreted with caution. Estimates are based on data from thousands of cases of this type of tumor, but the actual risk for a particular individual may differ.

Source: Central Brain Tumor Registry of the United States.

Find out more about basic cancer terms used in this section.

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A risk factor is anything that increases a person’s chance of developing a tumor. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do.

The cause of craniopharyngioma is not known. More than 50% of people with craniopharyngioma are younger than 18, with most cases occurring between the ages of 5 and 10. The second most common age range that craniopharyngioma occurs is between the ages of 40 and 60.

Children with craniopharyngioma may experience any of the symptoms listed below. Sometimes, children with craniopharyngioma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a tumor. If you are concerned about a symptom on this list, please talk with your child’s doctor.

Symptoms caused by craniopharyngioma can be general (caused by the tumor pressing on the brain), or specific (affecting the normal functioning of part of the brain or caused by pressure on nerves or blood vessels). Generally, craniopharyngioma is not diagnosed until symptoms begin.

General symptoms include:

• Headaches, which may be severe and may be worse in the early morning
  
  
• Nausea and/or vomiting
  
  
• Difficulty with balance
  
  
• Increased sleepiness or fatigue
  
  
• Mood or behavior changes

Specific symptoms include:

• Vision changes, blurriness, or loss of peripheral vision
  
  
• Excessive thirst
  
  
• Increased urination
  
  
• Slow or halted growth
  
  
• Excessive weight gain
  
  
• Early or delayed puberty

Doctors generally obtain a picture of the brain to diagnose a brain tumor, using the imaging tests described below. For most tumors, removing a sample of the tumor or the entire tumor, and then looking at it under the microscope is the only way to determine the type of tumor.

A physical examination and blood tests to check hormone levels may suggest that imaging tests are needed to look for craniopharyngioma. There are two main types of imaging tests used to find craniopharyngioma.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the brain with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the brain and spinal column. A contrast medium may be injected into a patient’s vein to create a clearer picture.

To learn more about what to expect during common diagnostic tests, read Cancer.Net: Tests and Procedures.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: Newly Diagnosed.

Craniopharyngioma rarely, if ever, spreads. There is no standard staging system for this type of tumor.

To take advantage of special expertise necessary to treat brain tumors, all children with brain tumors should be treated at a specialized pediatric center. Doctors at these centers have extensive experience in treating children with brain tumors and have access to the latest technology. Many times, a team of doctors treats a child with craniopharyngioma. Pediatric treatment centers often have extra support services for children and their families, such as nurse specialists, nutritionists, and social workers. Special activities for kids with cancer may also be available.

The type of treatment used depends on whether the tumor can be completely removed by surgery. If the tumor cannot be completely removed, radiation therapy is usually recommended. However, the side effects of radiation therapy must be considered when deciding on the best treatment for very young children, because it can cause age-related permanent learning and memory problems as well as lower levels of necessary hormones.

Surgery

Surgery is the most common treatment for craniopharyngioma. The goal of surgery is to confirm the diagnosis and completely remove the tumor, if possible.

Sometimes, the tumor may involve the optic chiasm (the area of the brain that controls vision) or involve major blood vessels, making removal of the tumor difficult. Depending on where the tumor is located, surgery may damage parts of the brain near the tumor, affecting hormone function, vision, arm and leg movement, or consciousness. Some tumors cannot be completely removed because of their location. These tumors are called inoperable. Complete removal of craniopharyngioma is possible for about 70% to 85% of patients.

Research studies have shown that people treated with partial resection (partial removal; where visible tumor is left behind after surgery) followed by radiation therapy have survival rates similar to those who had a complete removal of the tumor, with fewer side effects, such as stroke, hemorrhage (severe bleeding), or damage to the hypothalamus (a small structure above the pituitary gland that regulates the pituitary gland and many other body functions).

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill tumor cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. Because radiation therapy can interfere with the normal growth and development of a child’s brain, advanced treatment planning techniques should be used to reduce the amount of radiation to the areas of the brain not affected by the tumor.

Radiosurgery is a way to deliver a single, high dose of radiation therapy to the tumor and not to other areas of the brain. This technique requires a head frame to help to localize the tumor, so doctors know exactly where to deliver the radiation treatment.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. These side effects go away soon after treatment is finished. More permanent side effects include hair loss, learning differences, low hormone levels, weight gain, and memory problems.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: During Treatment.

Doctors and scientists are always looking for better ways to treat children with craniopharyngioma. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating craniopharyngioma. Even if they do not benefit directly from the clinical trial, their participation may benefit future children with craniopharyngioma.

To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about Clinical Trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.

A tumor and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.

Fear of treatment side effects is common after a diagnosis of a tumor, but it may be helpful to know that preventing and controlling side effects is a major focus of your child’s health-care team. Before treatment begins, talk with your child’s doctor about possible side effects of the specific treatments your child will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of tumor, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.

Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with your doctor about side effects your child experiences during and after treatment. For more information on the most common side effects of tumors and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects, based on ASCO’s curriculum.

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient.

For more information on late effects or long-term side effects, please read the After Treatment section or talk with your child’s doctor.

After treatment for craniopharyngioma ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children treated for craniopharyngioma should have life-long, follow-up care.

Follow-up care is important to determine whether the tumor is starting to grow again. It is also important to monitor the person’s visual, endocrine (hormone), and metabolic functions. As a result of the tumor and its treatment, hormone replacement therapy (medication used to replace hormones in the body when the body can no longer produce adequate amounts) is almost always necessary. Because of the frequent problems with slow metabolism and weight gain, a regular exercise program and dietary changes are often recommended.

A child treated for craniopharyngioma should have regular MRI scans to check for any growth or return (recurrence) of the tumor. Because craniopharyngioma is slow-growing and because the likelihood of tumor recurrence is low, MRI scans are often only done once or twice a year. If the patient has been treated with radiation therapy, there is a small possibility that a different type of brain tumor may develop years after the radiation therapy is given.

Based on the type of treatment the child received, the doctor will determine what examinations and tests are needed to check for long-term side effects and the possibility of secondary tumors. Your doctor can recommend the necessary screening tests. Follow-up care should also address the child’s quality of life, including any developmental or emotional concerns. Learn more about Childhood Cancer Survivorship<.

The child’s family is encouraged to organize and keep a record of the child’s medical information, so that as the child enters adulthood, he or she has a clear, written history of the diagnosis and details of the treatment given. The doctor’s office can help you compile this, and it should include recommendations from the doctor about the schedule for follow-up care. This information will be valuable to doctors who care for your child during his or her lifetime.

Children who have had a tumor can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: After Treatment.

Most children with craniopharyngioma are not treated in clinical trials if surgery can be done to remove all visible tumor, or if their age does not limit the use of radiation therapy. However, research on more advanced diagnostic procedures and treatments for craniopharyngioma is ongoing. Always discuss all diagnostic and treatment options with your child’s doctor.

Different methods of giving drugs. Interferon, chemicals, or chemotherapy injected directly into the tumor cyst (if there is a large one) is being evaluated for some patients. Interferon is a type of immunotherapy. Immunotherapy (also called biologic therapy) is designed to boost the body’s natural defenses to bolster, target, or restore immune system function.

Regular communication with your child’s doctor is important in making informed decisions about his or her health care. Consider asking the following questions of your child’s doctor:

• What type of tumor does my child have?
  
  
• Are other tests or surgery needed to confirm this diagnosis?
  
  
• Can you explain my child’s pathology report (laboratory test results) to me?
  
  
• What is your familiarity with craniopharyngioma and its treatment?
  
  
• How many CNS tumors do you treat each year?
  
  
• Do you attend meetings to discuss complicated tumor cases and possible new treatments for these tumors? What types of specialists attend such meetings?
  
  
• Will an experienced neuropathologist (a doctor who specializes in the diagnosis of diseases of the nervous system by studying tissue under a microscope) review my child’s pathology slides?
  
  
• What are the treatment options?
  
  
• What clinical trials are open to my child?
  
  
• What treatment do you recommend? Why?
  
  
• What are the possible side effects of this treatment, both in the short term and the long term?
  
  
• How will this treatment affect my child’s daily life? Will he or she be able to go to school and perform his or her usual activities?
  
  
• Should I get a second opinion on this treatment plan?
  
  
• Are there pediatric treatment centers that you recommend?
  
  
• What are the chances for success with the planned treatment?
  
  
• Does your practice include multidisciplinary care?
  
  
• Do you work with a social worker that assists patients with CNS tumors?
  
  
• Do you know of a local support group for patients with CNS tumors?
  
  
• Do you have reading material that would help me understand my child’s disease?
  
  
• Who answers questions when you are unavailable?
  
  
• What follow-up tests will my child need, and how often will he or she need them?
  
  
• What support services are available to my child? To my family?

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