Malignant melanoma with ectopic production of adrenocorticotropic hormone. Palliative treatment with inhibitors of adrenal steroid biosynthesis.

Abstract

A 56-yr-old woman developed severe Cushing's syndrome and recurrent malignant melanoma three years after the removal of the primary lesion. She had elevated plasma cortisol levels which showed no normal diurnal rhythm. Neither these nor her elevated urinary steroid excretion were suppressed by administration of high doses of dexamethasone. She had markedly elevated plasma immunoreactive ACTH levels. Bioactive and immunoreactive ACTH was present in an extract of the metastic tumor tissue. This is the first documented case of ectopic ACTH syndrome caused by malignant melanoma. The patient, who was moribund from the effects of severe hypercortisolism, was cured of her Cushing's syndrome by the combined administration of aminoglutethimide, metyrapone, and o,p'DDD. She was dramatically improved clinically, despite steady progression of tumor growth. It appears that the use of o,p'DDD, especially in combination with inhibitors of adrenal steroid biosynthesis, is an effective means for correcting hypercortisolis...