Key Points for This Section

Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland. There are no known risk factors for childhood craniopharyngioma. Possible signs of childhood craniopharyngioma include vision changes and slow growth. Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas. Childhood craniopharyngiomas are diagnosed and may be removed in the same surgery. Certain factors affect prognosis (chance of recovery) and treatment options.

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 ¹ 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 ² 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.
• 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.

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.
• Whether the tumor has just been diagnosed or has recurred (come back).

Glossary Terms

A hormone made in the pituitary gland. Adrenocorticotropic hormone acts on the outer part of the adrenal gland to control its release of corticosteroid hormones. More adrenocorticotropic hormone is made during times of stress. Also called ACTH and corticotropin.

Not cancer. Benign tumors may grow larger but do not spread to other parts of the body.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.

The growth of abnormal cells in the tissues of the brain. Brain tumors can be benign (not cancer) or malignant (cancer).

A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

A benign brain tumor that may be considered malignant because it can damage the hypothalamus, the area of the brain that controls body temperature, hunger, and thirst.

A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.

A sac or capsule in the body. It may be filled with fluid or other material.

The process of identifying a disease, such as cancer, from its signs and symptoms.

A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.

A metal element that is used in magnetic resonance imaging (MRI) and other imaging methods. It is a contrast agent, which helps show abnormal tissue in the body during imaging with a special machine.

An organ that makes one or more substances, such as hormones, digestive juices, sweat, tears, saliva, or milk. Endocrine glands release the substances directly into the bloodstream. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.

One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.

The area of the brain that controls body temperature, hunger, and thirst.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

Having to do with metastasis, which is the spread of cancer from one part of the body to another.

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.

A feeling of sickness or discomfort in the stomach that may come with an urge to vomit. Nausea is a side effect of some types of cancer therapy.

The removal of tissue or fluid with a needle for examination under a microscope. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.

A series of questions and tests to check brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk, and how well the muscles, sensory systems, and deep tendon reflexes work.

A procedure in which a surgical incision (cut) is made through the skin to expose and remove tissues. The biopsy tissue is examined under a microscope by a pathologist. An open biopsy may be done in the doctor’s office or in the hospital, and may use local anesthesia or general anesthesia. A lumpectomy to remove a breast tumor is a type of open biopsy.

The nerve that carries messages from the retina to the brain.

A part of the body that performs a specific function. For example, the heart is an organ.

A doctor who identifies diseases by studying cells and tissues under a microscope.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

An exam of the body to check for general signs of disease.

The main endocrine gland. It produces hormones that control other glands and many body functions, especially growth.

The original tumor.

The likely outcome or course of a disease; the chance of recovery or recurrence.

The time of life when a child experiences physical and hormonal changes that mark a transition into adulthood. The child develops secondary sexual characteristics and becomes able to have children. Secondary sexual characteristics include growth of pubic, armpit, and leg hair; breast enlargement; and increased hip width in girls. In boys, they include growth of pubic, face, chest and armpit hair; voice changes; penis and testicle growth, and increased shoulder width.

To come back or to return.

An abnormal mass of tissue that usually does not contain cysts or liquid areas. Solid tumors may be benign (not cancer), or malignant (cancer). Different types of solid tumors are named for the type of cells that form them. Examples of solid tumors are sarcomas, carcinomas, and lymphomas. Leukemias (cancers of the blood) generally do not form solid tumors.

The study of the amount of light that is taken up, given off, or scattered (reflected) by an object. Spectroscopy breaks down light and measures different wavelengths of visible and non-visible light. In medicine, different types of spectroscopy are being used to study tissues and to help make a diagnosis.

A type of paranasal sinus (a hollow space in the bones around the nose). There are two large sphenoid sinuses in the sphenoid bone, which is behind the nose between the eyes. The sphenoid sinuses are lined with cells that make mucus to keep the nose from drying out.

A column of nerve tissue that runs from the base of the skull down the back. It is surrounded by three protective membranes, and is enclosed within the vertebrae (back bones). The spinal cord and the brain make up the central nervous system, and spinal cord nerves carry most messages between the brain and the rest of the body.

The height of a person while standing.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A hormone produced by the pituitary gland. Thyroid-stimulating hormone stimulates the release of thyroid hormone from thyroglobulin. It also stimulates the growth of thyroid follicular cells. An abnormal thyroid-stimulating hormone level may mean that the thyroid hormonal regulation system is out of control, usually as a result of a benign condition (hyperthyroidism or hypothyroidism). Also called TSH.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.

Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.

A blood vessel that carries blood to the heart from tissues and organs in the body.

To eject some or all of the contents of the stomach through the mouth.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.