What is craniopharyngioma?

Craniopharyngioma is a type of benign brain tumor. It is called benign because the abnormal cells do not travel and grow in other parts of the body. Even though they don't spread to other parts of the body, they can cause problems by growing in the brain. They will press against the brain and cause issues.

This tumor results from an abnormal overgrowth of the part of the brain called Rathke's pouch. It is near the pituitary gland. The tumor is often partially a solid and partially fluid-filled cyst. It can contain calcium deposits and the fluid is made up of protein, lipid (fat), and cholesterol pieces. This growth can cause problems with hormone secretion and vision because of where it is in the brain.

What is craniopharyngioma and who is at risk?

Craniopharyngiomas occur in children and adults, most often in children ages 5-14 and adults ages 50-75. There are about 350 new cases diagnosed each year. The cause of these tumors is not known. Some researchers think that they begin to grow before birth, but it is not associated with any race, gender, or family history.

How can craniopharyngioma be prevented in a child?

Craniopharyngioma cannot be prevented because the cause of the tumor is not known.

What screening tests are used?

There are no screening tests for craniopharyngioma.

What are the signs and symptoms?

Symptoms of craniopharyngioma are caused by the pressure on the brain, nerves, and blood vessels from the growing tumor. Where in the brain the tumor presses determines the type of symptoms a child has. Often, there are changes to vision and a child's growth can be affected. Growth changes are related to changes in hormones from the tumor affecting the pituitary gland. In general, side effects can include:

Headache.
Changes in vision such as loss of peripheral vision and blurriness.
Nausea and vomiting with or without a headache.
New trouble walking or keeping balance.
Feeling more tired than usual.
Loss of hearing.
Not growing as fast as expected.
Change in the child's personality or how they act.
Unintended weight gain.
Feeling more thirsty than normal and peeing more than usual.

Each child can have different symptoms. If your child is having any changes in their normal activity, growth, or is having any changes in health, they should be seen by their provider.

How is childhood craniopharyngioma diagnosed?

A number of tests can be used to help diagnose craniopharyngioma:

To start, your child will have a full physical examination. The provider will ask you and your child about symptoms.
A vision exam will be done to see if there are any changes to the child's vision.
A neurological exam which includes questions and tests will be done. This checks how well the brain, spinal cord, and nerves are working.
Blood and urine tests may be done to check levels of hormones and to see how well the organs in the body are working.
Imaging tests, including a CT and MRI, may also be done to see what is going on inside the body.
Depending on the results of all of these tests, a biopsy may be done. A biopsy is the only definitive way to diagnose craniopharyngioma. A biopsy is the use of surgery or a procedure to remove part of or all of the tumor. A pathologist will look at the piece of the tumor under a microscope to make a diagnosis. A biopsy can be done in a few ways:
- A hollow needle may be placed through a hole in the skull and into the tumor to remove tissue. This is called an open biopsy. If the needle is guided by a computer then it is called a computer-guided needle biopsy.
- In some cases, a transsphenoidal biopsy is done. In this type of biopsy the tools needed are inserted into the nose and sphenoid bone. They are then guided into the brain to obtain tissue for a biopsy.
- In some cases, surgery is done to attempt to remove the whole tumor at one time. This is done both as a biopsy and treatment.

How is childhood craniopharyngioma staged?

Staging is often used in cancer to help create a treatment plan. Staging takes into account how large a tumor is, how it looks under a microscope, and if it has spread. There is no standard staging system for craniopharyngioma because it rarely spreads to other parts of the body. Some providers will describe it as newly diagnosed or recurrent.

How is childhood craniopharyngioma treated?

It is important to find a pediatric facility that has experience treating patients with craniopharyngioma. A team of specialists, along with you and your child, will determine the best treatment plan. A major factor when determining the treatment plan is whether the whole tumor can be removed with surgery. Something to consider when planning a treatment course is that there is potential for late or long-term side effects, meaning side effects that can occur after treatment has ended. Late effects of treatment for craniopharyngioma can include issues with vision, hormone imbalances, neurological issues, and issues with growth and weight gain. The provider should speak about these possible side effects.

Surgery

Surgery is the most common treatment used. The goal is to surgically remove the whole tumor but this is not always possible. If the tumor has grown into a major blood vessel or the optic nerve, or if the surgery itself could damage the brain and the child's functioning, then other treatments may be used. Other treatments may also be used if only part of the tumor is removed. The types of surgery used include:

An opening may be made by removing part of the skull. This is called a craniotomy. The tumor is removed through this opening.
Another type of surgery is called transsphenoidal. A cut is made under the upper lip or at the bottom of the nose between the nostrils. Through this cut tools are placed through the sphenoid bone and into the brain to remove the tumor.

The type of surgery used will depend on how big the tumor is and where it is in the brain. Your child's providers will talk to you about if surgery is right for your child and the method used.

Radiation

In some cases, radiation is done after surgery, especially if only part of the tumor was removed. Radiation is the use of high energy x-rays to kill cancer cells. Radiation can be used instead of surgery, after surgery, or if the tumor comes back. There is both internal and external radiation:

External radiation is radiation given from outside the body. The types used include stereotactic radiosurgery, intensity-modulated photon therapy, and proton therapy.
Internal radiation uses needles, seeds, wires, or catheters to insert radiation directly into the tumor. This is also called intracavitary radiation.

The type of radiation used will depend on each individual situation.

Chemotherapy

Chemotherapy is the use of medications that kill cancer cells. It can be given into a vein (IV), into a muscle, by mouth, into the cerebrospinal fluid, or into the tumor itself. Chemotherapy is often used to treat tumors that are not in the brain or in cases where the cancer has spread to different parts of the body. Intracavitary chemotherapy, or chemo injected directly into the tumor, can be used for a recurrent craniopharyngioma.

Immunotherapy

Immunotherapy is the use of medications that use the patient's own immune system to fight the cancer. Interferon-alfa is a type of immunotherapy and it can be injected into the cyst. This can be used to delay other treatment or in a tumor recurrence.

Clinical Trials

There are clinical research trials for most types of cancer, and every stage of the disease. Clinical trials are designed to determine the value of specific treatments. Trials are often designed to treat a certain stage of cancer, either as the first form of treatment offered, or as an option for treatment after other treatments have failed to work. They can be used to evaluate medications or treatments to prevent cancer, detect it earlier, or help manage side effects. Clinical trials are extremely important in furthering our knowledge of the disease. It is through clinical trials that we know what we do today, and many exciting new therapies are currently being tested. Talk to your provider about participating in clinical trials in your area. You can also explore currently open clinical trials using the OncoLink Clinical Trials Matching Service.

Follow-up Care and Survivorship

After treatment for childhood cancer, you will be followed closely to monitor for the cancer coming back, to manage ongoing side effects, and to transition to survivorship. At first you will have frequent appointments with providers and have ongoing tests to monitor your health. As time goes on, these visits and testing will become less frequent. The oncology team will discuss the plan for follow up.

What can you do to live a healthy life after treatment? There is no supplement or specific food you can eat to assure good health, but there are things you can do to live healthier, prevent other diseases, detect cancers early and work with the social and emotional issues, including insurance, employment, relationships, sexual functioning, and fertility, that a prior cancer diagnosis sometimes brings with it. Your oncology team is there to support you and can help you find support resources.

It is important to have a plan for who will provide follow up care (an oncologist, survivorship doctor or primary care doctor). Talk with the team about developing a survivorship care plan or develop one using the Smart ALACC tool. Your child may benefit from being seen in a survivorship clinic. At a survivorship clinic a provider can review your child's history and provide recommendations. You can contact cancer centers in your area to see if they have a survivor's clinic or search for a clinic on OncoLink's survivorship clinic list.

Resources for More Information

American Brain Tumor Association

Pediatric Brain Tumor Foundation

All About Craniopharyngioma