[MOL] ACTH Syndrome, long term treatment [01633] Medicine On Line

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[MOL] ACTH Syndrome, long term treatment

Long-term Medical Treatment of Ectopic ACTH Syndrome

Richard J. Comi, MD, Lebanon, NH, Phillip Gorden, MD, Department of Medicine, Dartmouth Hitchcock Medical Center, Lebanon, NH; and the Diabetes Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Md.


Background. The morbidity of hypercortisolemia due to ectopic production of ACTH by various tumors may be greater than the morbidity of the tumor itself.
Methods. We report three cases of long-term treatment of ectopic ACTH syndrome due to metastatic bronchial carcinoid, islet cell carcinoma, and malignant thymoma tumors. Clinical and biochemical eucortisolemia was achieved in each case and was sustained from 24 to 55 months. We review the therapeutic options and their reported efficacy.
Results. Cessation of therapy resulted in recurrence of hypercortisolemia in each case, showing the effectiveness of therapy.
Conclusion. Long-term treatment of ectopic ACTH-induced hypercortisolemia by blocking adrenal steroidogenesis is clinically effective and well tolerated. [South Med J 91(11):1014-1018, 1998. © 1998 Southern Medical Association]


Cushing's syndrome results from the ectopic production of corticotropin (ACTH) in about 15% of patients with peptide-producing tumors. In at least half of these patients, the ectopic ACTH syndrome is caused by a small cell carcinoma of the lung. In these patients, the prognosis is poor regardless of the therapeutic modality. By contrast, in non-small cell cases of ectopic ACTH syndrome, the tumors are much less aggressive, and control of the hypercortisolism becomes the paramount therapeutic issue. Whenever possible, these tumors should be removed surgically. However, in some patients the tumor is occult or metastatic, and resection is not possible.

We previously reported a case of severe hypercortisolism resulting from the ectopic ACTH syndrome in a patient who was effectively treated by medical therapy for more than 4.5 years.[1] In reviewing the more recent literature on the medical treatment of ectopic ACTH syndrome, we have realized that three of our patients are representative of the longest responders to the medical treatment of the ectopic ACTH syndrome. These three cases are herein reported and considered with the various medical therapies that are now available for the treatment of the ectopic ACTH syndrome.