title: Bilateral adrenocortical adenomas causing ACTH-independent Cushing's syndrome at different periods: a case report and discussion of corticosteroid replacement therapy following bilateral adrenalectomy.
abstract: We report a rare case of bilateral adrenocortical adenomas causing ACTH-independent Cushing's syndrome at different periods 9 yr apart. The subject, a 24-yr-old woman, in June 1989 had a typical Cushingoid appearance. Her baseline plasma cortisol levels did not show a diurnal rhythm and she had a very low baseline plasma ACTH level. Plasma cortisol levels could not be suppressed by overnight low-dose or two-day high-dose dexamethasone suppression test. Marked uptake of 131I-6beta-iodomethyl-19-norcholesterol (NP-59) was observed in the right adrenal gland. Abdominal computed tomography (CT) showed a right adrenal tumor. The right adrenal gland with adenoma was removed. The non-tumorous part of the adrenal cortex was atrophic. By April 1998, she had experienced a weight gain of more than 20 kg over a two-yr period. The baseline plasma cortisol levels were at the lower limit of the normal range with loss of diurnal rhythmicity. The baseline plasma ACTH levels were very low. Neither a two-day low-dose nor a two-day high-dose dexamethasone suppression test could suppress serum cortisol or urinary free cortisol levels. NP-59 adrenal scan revealed increased uptake of the left adrenal gland at 72 h after intravenous injection of the tracer. Abdominal CT and magnetic resonance imaging (MRI) all demonstrated a left adrenal mass. Left adrenalectomy was performed in June 1998; histological features showed a cortical adenoma and atrophic change in the non-tumorous part of the adrenal cortex. Elevated plasma ACTH levels after bilateral adrenalectomy could be suppressed with conventional corticosteroid replacement therapy and overnight low-dose dexamethasone suppression test.
authors.affiliation: Department of Internal Medicine, Division of Endocrinology and Metabolism, Kaohsiung Chang Gung Memorial Hospital, Taiwan. q0112323@ms26.hinet.net
authors.forename: S C
authors.initials: SC
authors.lastname: Tung
authors.forename: P W
authors.initials: PW
authors.lastname: Wang
authors.forename: T L
authors.initials: TL
authors.lastname: Huang
authors.forename: W C
authors.initials: WC
authors.lastname: Lee
authors.forename: W J
authors.initials: WJ
authors.lastname: Chen
mesh_terms: D000236:Adenoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D000302:Adrenal Cortex / Q000473:pathology; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000097:blood*; D000328:Adult; D002940:Circadian Rhythm; D003480:Cushing Syndrome / Q000209:etiology*; D003907:Dexamethasone; D005260:Female; D005938:Glucocorticoids; D020249:Hormone Replacement Therapy; D006801:Humans; D006854:Hydrocortisone / Q000097:blood / Q000652:urine; D008279:Magnetic Resonance Imaging; D009765:Obesity / Q000209:etiology; D014057:Tomography, X-Ray Computed
chemical_list: D000305:Adrenal Cortex Hormones; D005938:Glucocorticoids; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone