Long-term Medical Treatment of Ectopic ACTH SyndromeRichard J.
, Lebanon, NH, Phillip Gorden, MD
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.
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
Cessation of therapy resulted in
recurrence of hypercortisolemia in each case, showing the effectiveness of
Long-term treatment of ectopic
ACTH-induced hypercortisolemia by blocking adrenal steroidogenesis is clinically
effective and well tolerated. [South
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. 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.