Craniopharyngioma

What is a craniopharyngioma?

A craniopharyngioma is a rare benign tumor that develops near your pituitary gland. This is a slow-growing tumor that can affect your cranial nerves, which are responsible for vision, and your endocrine system, which is responsible for the hormonal function of your body.

The main treatments for craniopharyngiomas are surgery and radiation therapy. Chemotherapy has emerged as an option for a special papillary subtype of craniopharyngioma.

More than 90% of people with this tumor are alive five years after diagnosis. That said, these tumors are treated as a chronic condition because treatment doesn’t always cure the conditions the tumors cause and because this kind of tumor often comes back (recurs).

Who is affected by craniopharyngiomas?

Each year, approximately 2 people per 1 million are diagnosed with one of two types of craniopharyngiomas: adamantinomatous and papillary. They typically present in two age groups: in children aged 5 to 14 and in adults aged 50 to 74. Adamantinomatous types can be found in all age groups, whereas papillary subtypes are almost always found in adults.

How serious is a craniopharyngioma?

A craniopharyngioma is a serious medical condition that may require life-long medical treatment. About half of all surgically removed tumors come back over time. Craniopharyngiomas cause several medical conditions that remain even after the tumor has been removed.

What’s the difference between a craniopharyngioma and a pituitary adenoma?

Craniopharyngiomas and pituitary adenomas can both affect hormone function. Pituitary adenomas are tumors that come from your pituitary gland, and craniopharyngiomas are located near that gland. Although both tumors are considered benign, craniopharyngiomas generally have a more aggressive nature than pituitary adenomas.

What are craniopharyngioma symptoms?

Craniopharyngioma symptoms are linked to conditions that happen when something affects your or your child’s pituitary and hypothalamus glands, optic nerves and brain. In children, growth and development can be affected, which can be more obvious than when a craniopharyngioma develops in an adult. In both children and adults, vision can be affected, particularly parts of their peripheral vision. However, these symptoms can be similar to other conditions, making it difficult for healthcare providers to quickly conclude the symptoms are caused by this tumor.

What medical conditions are linked to craniopharyngioma pressure on my or my child’s pituitary?

Your or your child’s pituitary manages many hormonal functions. Pressure from a craniopharyngioma can cause the following conditions:

• Growth hormone (GH) deficiency: This condition affects children’s growth rates. Adults who have GH deficiency may have low energy, osteoporosis, little muscle strength, increased LDL cholesterol and heart problems.
• Gonadotropin deficiency: People with this issue often have delayed puberty. Women might not have periods, a condition known as amenorrhea.
• Adrenocorticotropic hormone (ACTH) deficiency: This condition causes people to feel weak and tired. Other conditions are weight loss, loss of appetite, muscle weakness, nausea and vomiting, and low blood pressure (hypotension).
• Thyroid stimulating hormone (THS) deficiency: People who have THS may have fatigue, irregular periods and forgetfulness.
• Central diabetes insipidus (CDI): This condition makes people feel extremely thirsty (polydipsia) and to pee a lot (polyuria.) People who have CDI can develop dehydration, irregular heartbeats, fever, dry skin and mucous membranes, confusion and seizures.

What medical conditions are linked to this tumor’s pressure on my or my child’s hypothalamus?

Your or your child’s hypothalamus controls body processes like mood, hunger and thirst, sleep patterns and sexual function. Craniopharyngioma pressure on your or your child’s hypothalamus sets the stage for the following conditions:

• Hypothalamic obesity: This is obesity that happens even after people change their eating and exercise habits by restricting how many calories they take in and by being more active.
• Froehlich’s syndrome: People with this condition often have obesity because they always feel hungry no matter how much they eat. Children with Froehlich’s syndrome may not develop as quickly as other children. Some children have poor vision and intellectual disabilities.
• Non-24-hour sleep-wake syndrome: This is a sleep disorder that prevents people from following a typical sleep schedule. Some people develop severe sleep deprivation.

How does this tumor affect my or my child’s vision?

Craniopharyngiomas often develop very close to your or your child’s optic nerves. Pressure on those nerves may blurred vision or peripheral vision problems.

What causes craniopharyngiomas?

Researchers haven’t pinpointed a cause, but they believe these tumors develop from cells that helped to form your or your child’s pituitary gland. Something causes these cells to transform into abnormal cells that multiply and grow.

How do healthcare providers diagnose craniopharyngiomas?

If you’re being evaluated for this kind of tumor, your healthcare provider may ask about your medical history, including your physical development. If your child is being evaluated, their healthcare provider may ask about their physical development, cognitive development, vision and whether they seem unusually thirsty or have to pee often.

What tests do healthcare providers do to diagnose craniopharyngiomas?

Healthcare providers may do the following tests:

• Blood tests: These tests measure certain hormone levels.
• Urinalysis: These tests check for signs of kidney problems.
• Computed tomography (CT) scan: This test uses a series of X-rays and a computer to create three-dimensional images of people’s soft tissues and bones.
• Magnetic resonance imaging (MRI): This is a painless test that uses a large magnet, radio waves and a computer to produce very clear images of organs and structures within people’s bodies.
• Biopsy: Healthcare providers do biopsies (take tissue samples) to obtain cells, fluids, tissues or growths for examination under a microscope.

Can craniopharyngiomas be treated?

Craniopharyngiomas are typically treated with brain surgery, which can be done either through your nose using a small scope, or through an opening in your head called a craniotomy. The goal of surgery is to remove as much of the tumor as possible while minimizing damage to your pituitary gland, hypothalamus or optic nerves. Even so, some people might need to take hormonal medications afterward. In addition, post-surgery radiation therapy may be recommended, especially if the entire tumor couldn’t be safely removed.

What are common surgery side effects?

Surgery to remove craniopharyngiomas can be very challenging. First, these tumors develop near delicate nerves and your pituitary gland, which are at risk for damage. Second, removing a craniopharyngioma is like pulling a sticky label off an envelope without tearing the envelope. That’s because these tumors tend to cling to your pituitary gland and the nerves that control vision. Some healthcare providers believe surgery to remove these tumors may cause as much damage as the tumors. So, it’s important to understand what the goal of surgery will be ahead of time and what side effects may be expected.

Can craniopharyngiomas be prevented?

These tumors can’t be prevented. These tumors develop due to changes in your or your child’s cells that happened while your or your child’s body was being formed.

How long can you live with craniopharyngioma?

Many people live for years after treatment. But these tumors often come back (recur). Overall, as many as 17% of tumors that are completely removed during surgery come back. That number increases to 25% to 63% for partially removed tumors. Most craniopharyngiomas that return will do so within three years after surgery.

Some healthcare providers consider a craniopharyngioma a chronic condition that needs long-term care and monitoring; here’s why:

• You or your child may need additional surgery to remove a recurring tumor.
• If the tumor or surgery caused problems with your or your child’s pituitary or hypothalamus, you or your child may need hormone replacement therapy.
• Many children who have tumor-related obesity, called hypothalamic obesity, have an increased risk for other health problems. If your child has hypothalamic obesity, you may need support and guidance so you can help your child make good food choices and get regular exercise.

When should I/my child see a healthcare provider?

Chances are you or your child will be coping with craniopharyngioma for years to come. These tumors tend to come back, and many times, treatment can’t make all symptoms go away. Here are some things to consider as you plan for your/your child’s care:

• You or your child will need to see healthcare providers annually so they can monitor your or your child’s overall health.
• You or your child’s healthcare providers likely will order annual scans, typically MRIs, to check for recurring tumors.
• You or your child probably will need help managing conditions like hormone deficiencies and vision problems that surgery didn’t cure.

What questions should I ask my/my child’s healthcare provider?

Craniopharyngiomas are rare benign tumors that can cause several challenging medical conditions that may require long-term care. If you or your child have this type of tumor, you may worry about managing this life-long condition. Some questions that may help you as you talk to your or your child’s healthcare provider include:

• I’ve never heard of this tumor. Can you explain what it is?
• Can this tumor cause me/my child to have more serious medical problems?
• Why do I/does my child have this tumor?
• What are the treatment options?
• What are the chances treatment will be successful?
• Will treatment make my or my child’s symptoms go away?
• What are treatment possible short-term and long-term side effects?
• What happens if the tumor comes back?

A note from Cleveland Clinic

Even after surgery to remove craniopharyngiomas, these rare benign tumors can have lasting effects on your or your child’s life. Moreover, you or your child may still need medical care for conditions the tumor caused. In a sense, a craniopharyngioma is a chronic illness that you or your child will need to manage for the rest of your lives. While healthcare providers can’t provide a cure, they can promise to do everything to help you and your child face the challenges of living with this rare tumor.