Brain & Nervous System Health Center

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%.[1] 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.[3] In cases in which hypercortisolemia persists, early repeat exploration and/or radiation therapy or laparoscopic bilateral adrenalectomy may be required.[2] Drug therapy is considered to be an adjunct to transsphenoidal microsurgery in cases with a residual tumor and in cases in which one is awaiting the effects of the radiation therapy.[1] 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.[4] 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.[1,5]

Standard treatment options:[1,2,3,4,5]

1. Surgery (the treatment of choice and usually a transsphenoidal approach).
2. Surgery plus radiation therapy.
3. Radiation therapy.
4. Steroidogenesis inhibitors, including mitotane, metyrapone, ketoconazole, and aminoglutethimide.

Treatment options under clinical evaluation:[5,6,7]

• Stereotactic radiation surgery.

Current Clinical Trials

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.

General information about clinical trials is also available from the NCI Web site.

References:

1. Yeh PJ, Chen JW: Pituitary tumors: surgical and medical management. Surg Oncol 6 (2): 67-92, 1997.
2. Levy A: Pituitary disease: presentation, diagnosis, and management. J Neurol Neurosurg Psychiatry 75 (Suppl 3): iii47-52, 2004.
3. Mampalam TJ, Tyrrell JB, Wilson CB: Transsphenoidal microsurgery for Cushing disease. A report of 216 cases. Ann Intern Med 109 (6): 487-93, 1988.
4. Nieman LK: Medical therapy of Cushing's disease. Pituitary 5 (2): 77-82, 2002.
5. Mahmoud-Ahmed AS, Suh JH: Radiation therapy for Cushing's disease: a review. Pituitary 5 (3): 175-80, 2002.
6. Devin JK, Allen GS, Cmelak AJ, et al.: The efficacy of linear accelerator radiosurgery in the management of patients with Cushing's disease. Stereotact Funct Neurosurg 82 (5-6): 254-62, 2004.
7. Wong GK, Leung CH, Chiu KW, et al.: LINAC radiosurgery in recurrent Cushing's disease after transsphenoidal surgery: a series of 5 cases. Minim Invasive Neurosurg 46 (6): 327-30, 2003.