Craniopharyngioma – Childhood

Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland.
Childhood craniopharyngiomas are rare tumors usually found near the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves).

Craniopharyngiomas are benign (not cancer) and do not spread to distant parts of the brain or to other parts of the body. However, they may grow into the pituitary gland, optic chiasm, optic nerves, or nearby fluid -filled spaces in the brain. Many functions including hormone making, growth, and vision may be affected. Craniopharyngiomas are usually part solid mass and part fluid-filled cyst.

This summary discusses the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary. See the PDQ treatment summary on Childhood Brain and Spinal Cord Tumors Treatment Overview for information about the different types of childhood brain and spinal cord tumors.

Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. (See the PDQ summary on Adult Brain Tumors for more information.)

There are no known risk factors for childhood craniopharyngioma.
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.

Possible signs of childhood craniopharyngioma include vision changes and slow growth.
These and other symptoms may be caused by craniopharyngiomas. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

• 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.

Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas.

The following tests and procedures may be used:

• 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.

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.

Certain factors affect 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.

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