Cushing's syndrome presenting the coexistence of a pituitary corticotrophic cell hyperplasia and a unilateral functional adrenal adenoma.
A very unusual case of Cushing's syndrome is presented. Most of the preoperative biochemical and radiological examinations were compatible with Cushing's syndrome owing to a right adrenal adenoma. Exceptional findings include normal concentrations of adrenocorticotrophin (ACTH) in plasma as well as a disturbance of its circadian rhythmicity and a significant adrenocortical responsiveness to exogenous ACTH. Secretory patterns of ACTH did not change even after right adrenalectomy. Studies in vitro revealed that the adenoma itself, but not the surrounding normal adrenal, was the source of cortisol secreted in response to ACTH. Post mortem examinations disclosed unexpectedly a hormonally inactive left adrenal adenoma and a focal hyperplastic lesion of the anterior pituitary with an ACTH concentration 53 times higher than that of the remaining tissue of the gland. It is a possibility that this case may have represented a transition between pituitary-dependent adrenocortical hyperplasia and adrenal adenoma to this date reported in only one similar case.
D000236:Adenoma / Q000150:complications*; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000378:metabolism; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000378:metabolism / Q000494:pharmacology; D002940:Circadian Rhythm; D003480:Cushing Syndrome / Q000150:complications* / Q000378:metabolism; D003907:Dexamethasone / Q000494:pharmacology; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D006965:Hyperplasia; D008797:Metyrapone / Q000494:pharmacology; D008875:Middle Aged; D010903:Pituitary Gland, Anterior / Q000473:pathology*; D010913:Pituitary-Adrenal System / Q000187:drug effects
D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone; D008797:Metyrapone