What is craniopharyngioma in children?

Craniopharyngioma is a brain tumor that’s not cancer (benign). A brain tumor is an abnormal growth of tissue in the brain. The brain is part of the central nervous system (CNS). The CNS also includes the spinal cord. The main parts of the brain are: 

• Forebrain. This part has the cerebrum. The cerebrum has two sides, the right and left hemispheres. It controls thoughts, emotions, speech, and movement. It also helps with understanding information from the senses. This includes seeing, hearing, smelling, touching, and feeling pain.

• Midbrain. This part organizes muscle movement and helps to maintain posture, balance, and equilibrium.

• Hindbrain. This part includes the midbrain, the pons, and the medulla. It is the part of the brain that connects to the spinal cord. It plays a part in controlling muscles and sensations from the body. Part of the brainstem helps control breathing and the heartbeat. Many of the cranial nerves start in the brain stem. The cranial nerves carry messages from different parts of the body to the brain.

Brain tumors start in the cells of the brain. They can be either of the following:                   

• Noncancer (benign). This kind of tumor doesn’t contain cancer cells. In most cases, once a benign tumor is fully removed, it often doesn’t grow back. Most benign brain tumors don’t grow into nearby tissue. These tumors can cause symptoms by pressing on the brain.

• Cancer (malignant). This kind of tumor does contain cancer cells. Malignant brain tumors usually grow fast and grow into nearby tissue. Malignant brain tumors don’t usually spread to other areas of the body. They may grow back after treatment.

Brain tumors can occur at any age. Brain tumors that occur in infants and children are very different from adult brain tumors.

What causes a craniopharyngioma in a child?

Most brain tumors are caused by abnormal genes or chromosomes. Researchers don’t know what causes them to be abnormal. Some chemicals may play a part in gene changes. Research is ongoing.

What are the symptoms of a craniopharyngioma in a child?

Symptoms can occur a bit differently in each child. The tumor can cause symptoms if it begins to grow into or press on an area of the brain. The most common symptoms are:

• Headache that may go away after vomiting

• Vision changes

• Loss of balance

• Trouble walking

• Unusual changes in energy level

• Slow growth or delayed puberty

• Hearing loss

• Weight gain

• Increase in thirst

• Increase in head size (in a baby)

• Mood or behavior changes

The symptoms of a craniopharyngioma can be like other health conditions. Have your child see a healthcare provider for a diagnosis.

How is a craniopharyngioma diagnosed in a child?

The healthcare provider will ask about your child's health history and symptoms. The provider will examine your child. This will include a neurological exam. The exam tests reflexes, muscle strength, eye and mouth movement, and coordination. Your child may also have tests, such as:

• Vision test. This is to check for changes in or problems with vision.

• CT scan. A CT scan uses a series of X-rays and a computer to make detailed pictures of the body.

• MRI. This test uses large magnets, radio waves, and a computer to make detailed pictures of the body. Contrast dye may be injected into your child's vein. It helps healthcare providers see tumor cells more clearly.

• Blood tests. Blood tests may be done to check levels of hormones, such as thyroid-stimulating hormone, prolactin, and adrenocorticotropic hormone. These are secreted by the pituitary gland.

How is a craniopharyngioma treated in a child?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how bad the condition is.

A craniopharyngioma is usually removed with surgery. In some cases, healthcare providers are not sure a tumor is a craniopharyngioma until surgery. The surgeon will see if the tumor can be removed fully. They may not be able to find this out before surgery.

In some cases, the surgeon isn’t able to remove all the tumor with surgery. This is because of the risk of damaging other tissues in the head. These include the optic nerve, hypothalamus, and carotid artery. A surgeon will remove as much of the tumor as possible. Your child may then need radiation therapy to shrink the rest of the tumor. This is usually done with external beam therapy. This radiation is pointed at the body from a machine. In some cases, your child may need experimental chemotherapy if the tumor grows back after radiation.

Talk with your child’s healthcare providers about the risks, benefits, and possible side effects of all treatments.

What are possible complications of a craniopharyngioma in a child?

The tumor can be fully removed with surgery in 9 out of 10 children. In some cases, there is a chance that the tumor will grow back, especially if all of it is not removed. Most cases of the tumor growing back happen within 2 years of surgery.

A child may have other health effects after treatment ends. Some common side effects of a craniopharyngioma include vision loss, obesity, behavioral problems, memory loss, and the need for lifelong hormone replacement. One common side effect is loss of posterior pituitary gland function. This causes uncontrolled urination. It can be partly treated with hormonal therapy.

When should I call my child’s healthcare provider?

Call the healthcare provider if your child has:

• Symptoms that don’t get better or get worse

• New symptoms

• Side effects from treatment

Key points about a craniopharyngioma in children

• A craniopharyngioma is a noncancer (benign) tumor that is found near the pituitary gland. The pituitary gland is connected to the brain. It controls the production of many hormones in the body.

• As the tumor grows, it may press on parts of the brain and nearby tissue. This affects hormones, vision, and other normal functions. 

• The most common symptoms are headaches that may go away after vomiting, vision changes, loss of balance, and trouble walking.

• Your child may have blood tests and imaging tests to diagnose the tumor. In some cases, the type of tumor isn’t known until surgery.

• Treatment may include surgery and radiation. There may be some complications after treatment.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.

• Before your visit, write down questions you want answered.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.

• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.

• Ask if your child’s condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if your child does not take the medicine or have the test or procedure.

• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your child’s healthcare provider after office hours. This is important if your child becomes ill and you have questions or need advice.