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What is Childhood Craniopharyngioma?
Craniopharyngioma is a type of central nervous system (CNS) tumor. A tumor begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous, slow-growing) or malignant (cancerous, usually faster-growing). Craniopharyngioma is a benign tumor that is usually slow-growing and unlikely to spread.
About the central nervous system
The brain and spinal cord make up the CNS, where all vital functions of the body are controlled. The brain is the center of thought, memory, and emotion. It controls the five senses (smell, touch, taste, hearing, and sight); movement; and other basic body functions, including consciousness, heartbeat, circulation, and breathing. The spinal cord consists of nerves that carry information from the body to the brain and from the brain to the body. When a tumor grows within the CNS, it can affect a person's thought processes and/or movements. A tumor in the CNS can also be challenging to treat because the tissue surrounding the tumor may be vital to the body's functioning.
Craniopharyngioma starts near the pituitary gland in a part of the brain called the suprasellar region. This region is the area of the brain just above the sella, the part of the skull where the pituitary gland is located. The pituitary gland is an important gland in the brain and is often called the “master endocrine gland” because it produces several different hormones that affect many bodily functions.
About craniopharyngioma
Craniopharyngioma is a slow-growing tumor that can grow for many years before being found. It can be solid and/or cystic (a closed pouch or sac that contains fluid). The solid part may contain areas of calcium that can easily be seen on a CT scan. The cystic part of the tumor may also contain some solid material or debris and often contains very high amounts of protein.
Symptoms and Signs
Children with craniopharyngioma may experience any of the symptoms or signs listed below. Sometimes, children with craniopharyngioma do not show any of these symptoms, or these symptoms may be caused by another medical condition that is not a tumor. If you are concerned about a symptom or sign on this list, please talk with your child's doctor.
Symptoms caused by craniopharyngioma can be general (caused by pressure building up in the brain), or specific (affecting the normal functioning of the part of the brain where the tumor is pressing on nerves or blood vessels). Generally, craniopharyngioma is not diagnosed until symptoms appear.
General symptoms include:
Headaches, which may be severe and may be worse in the early morning
Nausea and/or vomiting
Difficulty with balance
Increased sleepiness or fatigue
Mood or behavior changes
Location-specific symptoms include:
Vision changes, blurriness, or loss of peripheral vision
Excessive thirst
Increased urination
Slow or halted growth
Excessive weight gain
Early or delayed puberty
The doctor will ask you and your child questions about the symptoms your child is experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you've been experiencing the symptom(s) and how often.
If a tumor is diagnosed, relieving symptoms and side effects remains an important part of your child's care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with the health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.
Risk Factors
A risk factor is anything that increases a person's chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do.
The cause of craniopharyngioma is not known. The only known risk factor is age. More than 50% of people with craniopharyngioma are younger than 18, with most being diagnosed between the ages of 5 and 10. The second most common age range is between the ages of 40 and 60.
Diagnosis
In diagnosing craniopharyngioma, your child's doctor may consider different factors when choosing tests, including:
Child's age and medical condition
Type of tumor suspected
Severity of symptoms
Previous test results
The following tests may be used to diagnose craniopharyngioma:
Physical examination. The doctor will examine the patient's head and body and ask questions about the symptoms the patient is experiencing and his/her medical history. This may also include tests to check the patient's vision and CNS functioning.
Blood tests. The doctor may recommend different blood tests, including checking the levels of certain hormones, to help determine whether there is reason to look for a brain tumor.
Results of the physical examination and blood tests may suggest that imaging tests are needed to look for a craniopharyngioma. There are two main types of imaging tests used to find craniopharyngioma.
Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the brain with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient's vein to provide better detail.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the brain and spinal column. A contrast medium may be injected into a patient's vein to create a clearer picture.
Biopsy. A biopsy is the removal and examination of a small piece of tumor. A neurosurgeon is a doctor who specializes in CNS surgery. For craniopharyngioma, this means that a neurosurgeon removes a sample of the tumor or the entire tumor so that a pathologist can look at it under a microscope. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. A neuropathologist is a pathologist who specializes in CNS tissues and diseases. During the surgery, a small piece of tumor may be removed initially and processed by the pathologist so that the surgeon knows what kind of tumor it is. Most neurosurgeons will try to remove as much of the tumor as possible once they know that it is a craniopharyngioma.
Stages
Craniopharyngioma rarely, if ever, spreads. There is no standard staging system for this type of tumor.
