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Craniopharyngioma

What is craniopharyngioma?

Craniopharyngiomas are rare brain tumors. They usually develop near the pituitary gland and hypothalamus. Craniopharyngiomas grow slowly and rarely spread to other parts of the brain. 

These tumors are benign. But they can harm brain structures. Craniopharyngiomas affect endocrine function and hormone levels. They can affect vision if they press on the optic nerve. 

Craniopharyngiomas usually develop near the pituitary gland and hypothalamus. Tumors in this area affect endocrine function. If the tumor is near the optic nerve, vision may also be affected.

Craniopharyngiomas usually form near the pituitary gland and hypothalamus. Tumors in this area affect endocrine function. Vision may also be affected if the tumor is near the optic nerve.

Childhood craniopharyngioma is most common in children ages 5–14 years. The tumor is rare in children younger than 2.

Craniopharyngiomas account for about 6% of childhood brain tumors. There are around 100–120 cases of pediatric craniopharyngioma in the United States each year.

Treatments for craniopharyngioma include surgery, radiation therapy, or both. Surgery may:

  • Remove the full tumor
  • Remove part of the tumor
  • Relieve symptoms before radiation therapy

Radiation therapy can be used alone or with surgery.

The 10-year survival rate for craniopharyngioma is 80–90% in the United States. But long-term health problems are possible. This means it is important to work closely with your child’s care team.

Ongoing care can support your child’s quality of life.

Symptoms of craniopharyngioma

Signs and symptoms of craniopharyngioma can include:

  • Changes in hormones and endocrine function
  • Slowed growth
  • Delayed puberty
  • Vision problems or vision loss
  • Headache
  • Nausea and vomiting
  • Lower energy
  • Fatigue
  • Confusion
  • Problems in thinking and learning
  • Change in personality and behavior
  • Increased thirst or urination, which may be caused by diabetes insipidus
  • Low blood pressure
  • Weight gain
  • Increased or decreased appetite
What is hydrocephalus? As the tumor grows, it may block the normal flow of cerebrospinal fluid. This causes a build-up of fluid within the brain known as hydrocephalus.

As the tumor grows, it may block the flow of cerebrospinal fluid. This causes a build-up of fluid within the brain called hydrocephalus.

Craniopharyngioma with hydrocephalus

As it grows, the tumor may block the flow of cerebrospinal fluid. This causes a fluid build-up in the brain known as hydrocephalus. The fluid causes the pathways (ventricles) in the brain to widen. This increases pressure on the brain.

Many of the symptoms of craniopharyngioma are because of increased pressure in the brain.

Risk factors for craniopharyngioma

There are no known risk factors for craniopharyngioma.

Diagnosis of craniopharyngioma 

Tests to diagnose cancers such as craniopharyngioma may include:

  • Physical exam and health history 
  • Blood and urine tests to measure:
    • Hormones such as growth hormone, thyroid hormone, adrenocorticotropic hormone, and gonadotropin-releasing hormone (which controls the release of sex hormones)
    • Blood and urine levels of electrolytes such as sodium, calcium, and potassium
    • Urine output and concentration
    • Tumor markers, including alpha-fetoprotein and beta-human chorionic gonadotropin to rule out a germ cell tumor
  • A neurological exam to measure:
    • Memory
    • Vision
    • Hearing
    • Muscle strength
    • Balance
    • Coordination
    • Reflexes
  • Imaging tests, including:
  • Tumor tissue analysis from a biopsy or surgery 
  • Cyst fluid analysis from surgery
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Types of craniopharyngioma

There is no staging system for craniopharyngiomas. These tumors are divided into 2 types:

  1. Adamantinomatous craniopharyngioma is the most common type of craniopharyngioma in children. These tumors are irregularly shaped. They usually have cysts. They have areas of calcification. They often have a change in the CTNNB1 gene.
  2. Papillary craniopharyngioma is most common in adults. It is rare in children. These tumors appear more solid. The tumor cells usually have a change in the BRAF gene.

Treatment of craniopharyngioma 

Treatments include surgery and radiation therapy. 

Targeted therapy is being studied for some types of craniopharyngioma. Your child’s care team can talk with you about the risks and benefits of each treatment.  

  1. Surgery

    Most children with craniopharyngioma have surgery. Surgery may be used to:

    • Help in the diagnosis
    • Relieve symptoms 
    • Remove as much of the tumor as is safely possible

    The plan for surgery is based on the size and location of the tumor. The care team will consider potential risks, too. In some cases, long-term control is possible with surgery.

    There can be complications from surgery. This is because surgery may affect brain and endocrine functions.

    Doctors may plan limited surgery that does not fully remove the tumor. Goals of limited surgery may include:

    • Confirming the diagnosis
    • Draining fluid from the cyst in the tumor
    • Relieving pressure on the optic nerve

    Radical surgery involves removing the entire tumor. But sometimes this is not possible. In these cases, surgeons may perform a limited surgery in which they do not remove all of the tumor. Limited surgery is usually followed by radiation therapy.

    Radical surgery vs. limited surgery

      Pros Cons

    Radical surgery

    Avoid side effects linked to radiation therapy

    Surgery can damage nearby brain structures and cause both now and long-term:

    Acute injury, including stroke, vision loss, diabetes insipidus, hormone deficiencies, and traumatic brain injury

    Long term injury including personality changes, diabetes insipidus, hormone deficiencies, and obesity

    Limited surgery + radiation

    Less risk to personality

    Radiation therapy can cause long-term and late effects:

    • Cognitive effects, hormone deficiencies, hearing loss, blood vessel and tissue damage, stroke, and secondary tumors

    Different surgical methods are used to reach the tumor. The approach is based on the tumor's location and surgery goals.

    Surgery to manage cysts: A craniopharyngioma usually consists of a solid mass and fluid-filled cyst. In some cases, surgeons may place a catheter into the cyst to drain the fluid.

    Removing this fluid can help relieve symptoms. The catheter may be temporary or permanent. It might include the placement of an Ommaya reservoir.

    Surgery to manage hydrocephalus: Some patients may need surgery to help manage hydrocephalus. This may include creating an opening in the floor of the third ventricle in the brain. This is to keep fluid from building up.

    Doctors may place a shunt. This small tube drains cerebrospinal fluid from the brain. The shunt may be temporary or permanent.

  2. Radiation therapy

    Radiation therapy may be used after surgery. The type of radiation therapy depends on the tumor’s location. The care team may also consider your child’s age.

    Radiation therapy is used if the tumor comes back after an earlier radical surgery. Doctors will need to watch for late effects after your child’s treatment.

    Proton therapy may be a good choice in some cases. It is still being studied and is not available at every medical center.

    Radiation therapy is often given in small doses. Patients usually have treatments 5 days a week for about 6 weeks.

    A single dose of radiation therapy (radiosurgery) may be used in special cases.

    Radiation therapy is often used in addition to surgery for craniopharyngioma, especially if limited surgery was performed. This therapy uses beams of radiation to shrink tumors and kill cancer cells. Radiation works by damaging the DNA inside cancer cells.

    Radiation therapy is often used with surgery. This therapy uses beams of radiation to shrink tumors and kill cancer cells. Radiation works by damaging the DNA inside cancer cells.

Prognosis for craniopharyngioma 

The 10-year survival rate for craniopharyngioma is about 80–90%.

Your child’s care team is the best source of information about your child’s case.

Craniopharyngiomas may cause changes in endocrine function or damage the optic nerve. Long-term quality of life is important when planning treatments.

Support for craniopharyngioma

Most survivors of craniopharyngioma live with complications. These may include:

  • Endocrine system problems
  • Abnormal hormone levels
  • Obesity
  • Metabolic syndrome
  • Diabetes insipidus
  • Sleep disorders, including excessive daytime sleepiness (EDS)
  • Loss of vision
  • Changes in personality, emotions, or behaviors
  • Damage to blood vessels in the brain 
  • Disorders such as aneurysm and stroke
  • Problems with learning and memory
Life after craniopharyngioma may include problems with the endocrine system. The endocrine system is a group of glands that controls many of the body’s functions such as growth, puberty, energy level, urine production, and stress response.

Craniopharyngioma may cause problems with the endocrine system. The endocrine system is a group of glands that controls many of the body’s functions.

Hormone imbalances after craniopharyngioma

Life after craniopharyngioma may include problems with the endocrine system. The endocrine system is a group of glands that control many of the body’s functions, such as:

  • Growth
  • Puberty
  • Reproduction
  • Metabolism
  • Urine production
  • Sleep
  • Body temperature
  • Emotions
  • Responses to stress

Most problems after craniopharyngioma are related to abnormal hormone levels. These are caused by damage to the pituitary gland or hypothalamus.

Hormones that may be affected include:

  • Growth hormone
  • Thyroid stimulating hormone (TSH)
  • Adrenocorticotropic hormone (ACTH)
  • Follicle-stimulating hormone (FSH)
  • Luteinizing hormone (LH)
  • Prolactin
  • Antidiuretic hormone (ADH)

Your child may need hormone replacement therapy.

Pituitary Disorders: The pituitary gland is called the “master gland.” It controls many hormones and other glands. Abnormal pituitary function can lead to problems including:

  • Obesity
  • Poor lipid profile
  • Low bone mineral density
  • Fertility problems

These problems can happen even if your child takes hormone replacement medicines.

Hypothalamus Disorders: Damage to the hypothalamus causes hormone changes. Problems can include:

  • Increased appetite
  • Weight gain
  • Severe obesity

Changes in behavior and emotions are also common. They can include:

  • Mood swings
  • Loss of interest in activities
  • Social withdrawal
  • Fatigue

Obesity after craniopharyngioma

Craniopharyngioma often causes hypothalamic obesity. That means extreme weight gain caused by damage to the hypothalamus. This may be caused by:

  • Changes in metabolism (fewer calories burned at rest)
  • Increased appetite
  • Low physical activity levels

Obesity can happen even if your child does not eat more than normal. It is hard to treat hypothalamic obesity.

Obesity increases risk for heart disease, diabetes, stroke, and metabolic syndrome. It can also increase the risk for poor physical function, joint problems, and low fitness levels.

This makes healthy habits like good nutrition and physical activity even more important for survivors.

Metabolic syndrome after childhood craniopharyngioma

Metabolic syndrome is a common health issue. It can affect the quality of life for survivors of childhood craniopharyngioma.

This syndrome is a group of conditions that include:

  • Obesity
  • High blood pressure
  • High blood sugar levels
  • High triglyceride levels
  • Low HDL levels (good cholesterol)

Metabolic syndrome is linked to heart disease, stroke, and diabetes.

Sleep problems after craniopharyngioma

Sleep disorders such as excessive daytime sleepiness are common among craniopharyngioma survivors. They are often caused by damage to the hypothalamus. 

It is important for your child to follow healthy sleep habits. Talk with your child’s care team if they have sleep problems.

Brain health after craniopharyngioma

Treatments for craniopharyngioma can affect long-term brain health.

Survivors can be at higher risk of problems in brain blood vessels. These changes can sometimes lead to ischemia or stroke. Patients are also at increased risk for seizures.

Cognitive late effects may occur, too.

Classroom accommodations and school support services can help students and families cope with any educational challenges.

Other tips for families

There are things you can do to help promote your child’s quality of life:

  • Seek help from your care team: Children with craniopharyngioma need ongoing care. Care team members from psychology, child life, and social work can help with issues that arise.
  • Encourage healthy eating and exercise: A healthy diet and regular physical activity are important. These habits can also help improve sleep and learning. A nutrition professional can help. A licensed rehabilitation provider or a certified fitness professional can help plan exercise.
  • Promote good sleep habits: Sleep disorders are common in cancer survivors. A doctor may prescribe medicine to help with alertness. Or they may suggest therapy to improve sleep habits and develop coping skills. A healthy sleep routine is important.

Questions to ask your child’s care team

  • What are our treatment options? 
  • What are the possible side effects of each treatment? 
  • What can be done to manage my child’s symptoms? 
  • Will my child need to be in the hospital for treatment? 
  • What should we do to help my child have a higher quality of life for my child?

Key points about craniopharyngioma

  • Craniopharyngiomas are rare brain tumors. They usually develop near the pituitary gland and hypothalamus.
  • Craniopharyngiomas are benign and grow slowly. They rarely spread to other parts of the brain.
  • Because of their location, craniopharyngiomas affect endocrine function and hormone levels.
  • Craniopharyngiomas can affect vision if they compress the optic nerve.
  • Treatments for craniopharyngioma include surgery and radiation therapy. Sometimes both are used.
  • The 10-year survival rate for craniopharyngioma is about 80–90%.
  • Many survivors have late effects. This means regular care from your child’s care team will be vital.

—
Reviewed: August 2023

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