Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland.
Childhood craniopharyngiomas are rare tumors usually found near the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves).
Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other...
Steroidogenesis inhibitors, including mitotane, metyrapone, ketoconazole, and aminoglutethimide.[1,2,5]
For patients with corticotroph adenomas, transsphenoidal microsurgery is the treatment of choice.[1,2] Remission rates reported in most series are approximately 70% to 90%. In a series of 216 patients, who were operated on using a transsphenoidal approach, 75% experienced long-term remission, 21% experienced persistence of Cushing disease, and 9% had recurrence after the initial correction of the hypercortisolism. The average time interval for reoperation was 3.8 years. Seventy-nine percent of the tumors were microadenomas, and 18% were macroadenomas; 86% of the cases with microadenoma had long-term remission, whereas, only 46% of those with macroadenoma had remission. In cases in which hypercortisolemia persists, early repeat exploration and/or radiation therapy or laparoscopic bilateral adrenalectomy may be required.
Radiation therapy has been used in patients who are deemed to be poor surgical candidates and has also been used as adjunctive therapy in patients with residual or recurrent active tumor.[1,4]
Drug therapy is considered to be an adjunct to transsphenoidal microsurgery in cases in which there is a residual tumor and in cases in which one is awaiting the effects of the radiation therapy. Steroidogenesis inhibitors, including mitotane, metyrapone, ketoconazole, and aminoglutethimide are used. Ketoconazole is the best tolerated of these agents and is effective as monotherapy in about 70% of patients.
If untreated, patients frequently succumb to cardiovascular disease or infection.
Treatment Options Under Clinical Evaluation for ACTH-Producing Pituitary Tumors
Treatment options under clinical evaluation for ACTH-producing pituitary tumors include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with pituitary tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.