Craniopharyngiomas are tumors that develop near the pituitary gland at the base of the skull. They account for 2% to 4% of all brain tumors. Most grow slowly and are benign (not cancerous). They can spread (metastasize), however. They can occur at any age but most often affect children 5 to 10 years old, boys and girls equally, and adults 65 to 74 years old.
The cause is unknown. They are thought to grow from groups of special cells commonly found in the part of the brain called the suprasellar area surrounding the pituitary gland.
Tumors usually start slowly. People usually have no symptoms in early stages. They commonly have symptoms that progress slowly for 1 to 2 years. Symptoms are usually related to where the tumor is. Most people report headaches and have problems with vision. The usual problem is loss of side vision, called bitemporal hemianopsia. Other symptoms include mental changes, nausea, vomiting (especially in the morning), balance problems, and sleepiness. Damage to the pituitary can lead to lack of hormones. Adults often have sexual dysfunction. Men can be impotent. Women can stop having their periods. Urinating too often with large volumes of urine occurs in 25% of cases. It’s due to not enough antidiuretic hormone (diabetes insipidus), as the tumor grows and destroys pituitary tissue. These tumors may cause children to be very short (dwarfism), because of loss of pituitary cells that make growth hormone. Other endocrine problems can be precocious puberty in children and obesity in adults. Large tumors in adults can cause neuropsychiatric symptoms, memory loss, apathy, incontinence, depression, and lethargy.
The location of the tumors can make them hard to diagnose. The health care provider makes a preliminary diagnosis from the medical history, physical examination, and laboratory tests that measure hormone levels. Magnetic resonance imaging (MRI) or computed tomography (CT) of the brain will be done if craniopharyngioma is suspected. Special tests will include measuring vision loss (visual field evaluation). The health care provider will recommend seeing specialists (endocrinologist, neurosurgeon).
The main treatment is surgery to remove the tumor. Transsphenoidal surgery is used for small intrasellar tumors. Most people have subfrontal craniotomy. The goal is total tumor removal but making sure that the vision and pituitary structures (stalk) are preserved. Radiation therapy usually follows surgery. MRI is used after surgery to determine if the entire tumor has been removed. External beam radiotherapy is used for treatment of any remaining tumor.
The 5-year survival after treatment is 55% to 85%. When the tumor isn’t completely removed, the condition may recur, usually within the first year. People can have lasting hormonal, visual, and neurological problems after treatment and will need close follow-up and medications from an endocrinologist.
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Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.
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