Treatment Options for Adrenocorticotropic Hormone (ACTH)–Producing Pituitary Tumors

Treatment options for ACTH-producing pituitary tumors include:

Transsphenoidal microsurgery is the treatment of choice for patients with corticotroph adenomas.[1,2] Most series report remission rates of approximately 70% to 90%.[1] In a series of 216 patients who underwent surgery 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] 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, compared with only 46% of those with macroadenoma. In cases in which hypercortisolemia persists, early repeat exploration and/or radiation therapy or laparoscopic bilateral adrenalectomy may be required.[2]

Radiation therapy has been used in patients who are not surgical candidates and has also been used as adjuvant therapy in patients with residual or recurrent active tumor.[1,4]

Drug therapy may be an adjunct to transsphenoidal microsurgery in cases of residual tumor and in cases where radiation therapy has a delayed effect.[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.[5]

If untreated, patients frequently die of cardiovascular disease or infection.