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Craniopharyngiomas are benign (noncancerous) brain tumors that are slow-growing and unlikely to spread. Only in very rare cases do craniopharyngiomas become cancerous
Craniopharyngiomas are benign (noncancerous) brain tumors that are slow-growing and unlikely to spread. Only in very rare cases do craniopharyngiomas become cancerous.
What is a craniopharyngioma?
A craniopharyngioma is a rare type of benign central nervous system (CNS) tumor that forms near the pituitary gland and hypothalamus, which is the part of the brain that regulates sleep, growth, and reproductive functions.
It is estimated that craniopharyngioma accounts for approximately 6% of all brain tumors in children. The tumor is also called Rathke’s pouch tumor and craniopharyngeal duct tumor.
What are different types of craniopharyngioma?
There are two types of craniopharyngioma:
- Adamantinoma variant: Most common in children and forms in the cells in an embryologic structure called craniopharyngeal duct.
- Papillary variant: More common in adults and forms in the cells in the anterior part of the pituitary gland.
Both types of craniopharyngioma can be either solid or cystic:
- Solid: Contains areas of calcium that are easily visible on computed tomography (CT) scan.
- Cystic: Has a closed pouch or sac that contains fluid produced by the tumor, which often contains high amounts of protein.
What are the symptoms of craniopharyngioma?
Craniopharyngiomas may not cause symptoms for many years. However, symptoms of may develop due to:
- Hormonal changes: Pressure on the pituitary gland, which is responsible for the production of hormones needed for the regulation of various body functions.
- Pressure building up in the brain: Pressure on the hypothalamus, which regulates hunger, body temperature, thirst, sleep, fatigue, and other behaviors.
- Tumor pressing on nerves or blood vessels: Pressure on the optic nerve, leading to visual defects.
- Increased intracranial pressure: Compression of the foramen of Monro (a channel that helps circulate cerebrospinal fluid within the brain) can lead to the accumulation of the cerebrospinal fluid.
Craniopharyngioma symptoms in children include the following:
- Generalized
- Location-specific
- Obesity or excessive weight gain (hypothalamic obesity)
- Excessive thirst
- Increased frequency of urination
- Vision changes, blurred vision, or loss of peripheral vision
- Slow body growth
- Early or delayed puberty
- Disruption of sleep cycles
- Hydrocephalus (enlarged head due to excessive CSF accumulation)
- Paresis (limb weakness)
- Seizures
How is craniopharyngioma diagnosed?
Craniopharyngioma can be diagnosed at any age but is most often diagnosed between the ages of 5-14 or the ages of 50-74. Tests used to diagnose the tumor may include:
- Physical examination: Your doctor will perform a physical exam and ask you about your symptoms and medical history.
- Blood and urine tests: Different blood tests may be ordered, including evaluation of serum electrolytes and certain hormone levels, such as growth hormone, insulin-like growth factor 1, thyroid stimulating hormone, free thyroxine, cortisol, follicle stimulating hormone, luteinizing hormone, testosterone, estradiol, and prolactin.
- Biopsy: Biopsy involves the removal and lab analysis of tumor tissue and is the only definitive way to confirm a craniopharyngioma diagnosis.
- Imaging tests: The need for imaging tests depends on the results of the physical examination and lab tests and may include:
- Computed tomography scan: Three-dimensional images are produced by taking X-ray pictures of the brain from different angles.
- Magnetic resonance imaging: Magnetic fields are used to produce detailed images of the brain and spinal cord.
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How is craniopharyngioma treated?
Treatment of craniopharyngioma in children involves a team of doctors including a pediatric oncologist and a pediatric neuro-oncologist. Treatment may consist of:
- Surgery: Surgery aims to confirm the diagnosis and remove as much of the tumor as possible or remove it entirely.
- Radiation therapy: High-energy X-rays are used to destroy tumor cells.
- External-beam radiation therapy: Radiation given from a machine outside the body.
- Radiosurgery: A single, high dose of radiation therapy is delivered to the tumor while sparing other areas of the brain.
Craniopharyngioma and its treatment can lead to physical and emotional side effects that can be managed with medication, dietary changes, relaxation techniques, and other therapies and supportive care.
The 5-year survival rate for children with craniopharyngioma under age 15 is over 95%.
What are complications of craniopharyngioma?
For many children with craniopharyngioma, obesity is the most severe long-term side effect of craniopharyngioma due to an increased and uncontrolled appetite.
Other complications may include:
- Stroke
- Cardiac complications
- Respiratory complications
- Chronic hypothalamic insufficiency
- Hormonal deficiencies
- Seizures
In very rare cases, craniopharyngiomas can become cancerous.
