Craniopharyngioma

Information about pediatric craniopharyngioma, clinical trials, and research from Texas Children's Cancer Center. The Texas Children's Cancer Center Brain Tumor Program and the Neuro-oncology Team treats patients with craniopharyngiomas.

What are childhood craniopharyngiomas?

Childhood craniopharyngioma's are a type of childhood brain tumor that develops above the pituitary gland, which is found in the middle of the brain, behind the back of the nose. The pituitary is a pea-sized gland that controls vital body functions such as growth. Craniopharyngioma's are benign and rarely spread. However, they can have serious effects on important areas of the brain, such as the pituitary gland. Childhood craniopharyngiomas are rare tumors usually found near the pituitary gland. The pituitary gland is a pea-sized organ at the bottom of the brain that controls other glands. The hypothalamus is connected to the pituitary gland by nerves. It is a small cone-shaped organ. Craniopharyngiomas are usually part solid mass and part fluid-filled cyst. They are benign, which means non-cancerous. They do not spread to distant parts of the brain or to other parts of the body. However, they may grow and press on parts of the brain or other nearby areas, including the pituitary gland, the optic chiasm, optic nerves, and fluid-filled spaces in the brain. Many functions including hormone making, growth, vision, and normal working of the brain may be affected. Benign brain tumors need treatment. 

What are the risk factors for childhood craniopharyngioma?

There are no known risk factors for childhood craniopharyngioma. 

How common are childhood craniopharyngiomas?

Craniopharyngiomas are rare in children younger than 2 years of age and are most often diagnosed in children aged 5 to 14 years. It is not known what causes these tumors to form. 

What are the symptoms of a craniopharyngioma?

Possible signs of childhood craniopharyngioma include vision changes and slow growth. Other signs and symptoms include: 

• Headaches, including morning headache or headache that goes away after vomiting
• Vision changes
• Nausea and vomiting
• Loss of balance or trouble walking
• Increase in thirst or urination
• Increase in head size (in infants)
• Unusual sleepiness or change in energy level
• Unusual changes in personality or behavior
• Short stature, slow growth, or delayed puberty
• Hearing loss

How are childhood craniopharyngiomas diagnosed?

Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas. Tests and procedures that your doctor may use to diagnose this disease include: 

Physical exam and history. An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

Neurological exam. A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.

Visual field exam. An exam to check a person’s field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). Any loss of vision may be a sign of a tumor that has damaged or pressed on the parts of the brain that affect eyesight.

Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.

Blood hormone studies. A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. For example, the blood may be checked for unusual levels of thyroid-stimulating hormone (TSH) or adrenocorticotropic hormone (ACTH). TSH and ACTH are made by the pituitary gland in the brain.

CT scan (CAT scan). A procedure that makes a series of detailed pictures of areas inside the brain, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help areas of the brain show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

MRI (magnetic resonance imaging) of the brain and spinal cord with gadolinium. A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the tumor cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). Sometimes magnetic resonance spectroscopy (MRS) is done during the same MRI scan to look at the chemical makeup of the brain tissue.

Childhood craniopharyngiomas are diagnosed and may be removed in the same surgery. Doctors may diagnose a craniopharyngioma based on where the tumor is in the brain and how it looks on a CT scan or MRI. Sometimes a biopsy is required to find out the type of tumor. The biopsy is done during surgery, and a tissue sample is removed, using one of the following procedures:

• An open biopsy (a hollow needle is inserted through a hole in the skull into the brain).
• A computer-guided needle biopsy (guided by a computer, a hollow needle is inserted through a small hole in the skull into the brain).
• A transsphenoidal biopsy (instruments are inserted into part of the brain by going through the nose and the sphenoid bone, a butterfly-shaped bone at the base of the skull).
• A pathologist views the tissue under a microscope to look for tumor cells. If tumor cells are found, the doctor may remove as much tumor as safely possible during the same surgery.

What is the prognosis (chance of recovery) and treatment options?

The prognosis (chance of recovery) and treatment options depend on the following: 

• The size of the tumor
• Where the tumor is in the brain
• Whether the tumor is solid or a fluid-filled cyst
• Whether there are tumor cells left after surgery
• The child's age
• Whether the tumor has just been diagnosed or has recurred (come back)

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