Central nervous system atypical teratoid/rhabdoid tumor is a disease in which malignant (cancer) cells form in the tissues of the brain.
Central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT) is a very rare, fast-growing tumor of the brain and spinal cord. It usually occurs in children younger than three years of age, although it can occur in older children and adults.
About half of these tumors form in the cerebellum or brain stem. The cerebellum is the part of the brain that controls...
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.
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.
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Levy A: Pituitary disease: presentation, diagnosis, and management. J Neurol Neurosurg Psychiatry 75 (Suppl 3): iii47-52, 2004.
Mampalam TJ, Tyrrell JB, Wilson CB: Transsphenoidal microsurgery for Cushing disease. A report of 216 cases. Ann Intern Med 109 (6): 487-93, 1988.
Mahmoud-Ahmed AS, Suh JH: Radiation therapy for Cushing's disease: a review. Pituitary 5 (3): 175-80, 2002.
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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.
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.