Craniopharyngiomas are rare benign tumors. Less than 400 new cases are diagnosed in the U.S. each year. They may be located within the pituitary gland or above the pituitary gland. People with this condition are born with it, and the tumor may enlarge at any time.
These tumors generally come to attention either during childhood (5-15 years of age) or late adulthood (50-75 years of age). They are typically not diagnosed until they press on important structures around them, causing symptoms.
Although craniopharyngiomas are benign tumors (not cancerous), their location can make them difficult to remove completely. A multidisciplinary approach is essential to the appropriate management and treatment of patients with craniopharyngiomas.
Craniopharyngiomas can cause a wide variety of symptoms, but unlike pituitary adenomas, this type of tumor does not produce hormones. It does frequently interfere with normal pituitary gland function and may cause pituitary deficiencies.
- Increased cranial pressure
- Visual disturbances, including double vision and blurring
- Vomiting without nausea
- Altered level of consciousness
- Back pain
- Pituitary dysfunction and deficiencies
- Hypogonadism (decreased libido, altered menstrual cycles)
- Diabetes Insipidus (a disorder of water balance with frequent urination and excessive thirst)
- High blood pressure
An endocrinologist, neurosurgeon and neuro-ophthalmologist are often involved in the evaluation.
Tests and positive results include:
- Pituitary hormone blood tests
- CT and MRI scans
- Visual field and acuity testing: Due to the size and location of craniopharyngiomas, visual abnormalities often arise. While visual disturbance does not confirm a diagnosis of a craniopharyngioma, a detailed exam is important to assess vision and the health of the optic nerves.
- Cognitive testing: Performed by a neuropsychologist.
Most patients require hormone replacement(s) because of damage to the normal pituitary gland by the tumor.
Experts usually recommend complete surgical removal of the craniopharyngioma using either a craniotomy or a transsphenoidal approach.
Depending on the patient's age and the location of the remaining tumor, your surgeon may recommend a more conservative approach using radiation therapy or radiotherapy.
In selected cases of predominately cystic tumors, some patients may be treated with a less invasive drainage method, followed by a radioactive isotope or antineoplastic agent.
Evidence suggests that radiation therapy and radiosurgery provide effective tumor control in people with incompletely removed craniopharyngiomas.
Gamma Knife Radiosurgery
Gamma Knife delivers radiation in a more precise and accurate fashion than conventional radiation.
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Content was created using EBSCO’s Health Library. Edits to original content made by Rector and Visitors of the University of Virginia. This information is not a substitute for professional medical advice.