A craniopharyngioma is a benign (noncancerous) tumor arising from small nests of cells near the pituitary stalk. Crangiopharyngiomas are localized tumors and become large before they are diagnosed. How malignant they are and how quickly they are likely to spread are unknown.
Adamantinomatous (ordinary) craniopharyngioma occurs in children and tends to be less solid than papillary craniopharyngioma. Papillary craniopharyngioma occurs in adults and is a more solid tumor.
Craniopharyngiomas occur in the sellar region of the brain, near the pituitary gland. They often involve the third ventricle, optic nerve, and pituitary gland.
Increased pressure within the brain causes many of the symptoms associated with this tumor. Other symptoms result from pressure on the optic tract and pituitary gland. Obesity, delayed development, impaired vision, and a swollen optic nerve are common.
Surgery to remove the tumor is usually the first step in treatment. If hydrocephalus (excess water in the brain) is present, a shunt (drainage system) may be placed during surgery. The shunt will help remove excess cerebrospinal fluid from the brain and ease the pressure.
Radiation therapy may be suggested if all visible tumor cannot be removed. In children younger than 3, radiation may be delayed by the use of surgery or hormone therapies.
This tumor tends to be located close to the pituitary gland, which controls hormone balance in the body. To ensure the best outcome, an endocrinologist (a doctor trained to treat hormone imbalances) may work with the treatment team to develop a long-term care plan.
Craniopharyngiomas represent 2-5% of all primary brain tumors, and 5-10% of all childhood brain tumors. This tumor tends to be found in two age groups—patients up to age 14 and patients over age 45. They are more common in African-American patients.