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Bilateral adrenocortical adenomas causing ACTH-independent Cushing’s syndrome at different periods: A case report and discussion of corticosteroid replacement therapy following bilateral adrenalectomy

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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-6ß-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.

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Authors and Affiliations

  1. Department of Internal Medicine, Division of Endocrinology and Metabolism, Kaohsiung Chang Gung Memorial Hospital 123, Ta-Pei Road, Niao-Sung Hsiang, Kaohsiung Hsien, 83305, Taiwan

    S. -C. Tung & P-W. Wang

  2. Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Taiwan

    T-L. Huang

  3. Department of Surgery, Kaohsiung Chang Gung Memorial Hospital, Taiwan

    W-C. Lee

  4. Department of Pathology, Kaohsiung Chang Gung Memorial Hospital, Taiwan

    W-J. Chen

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  1. S. -C. Tung
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  2. P-W. Wang
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  3. T-L. Huang
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  4. W-C. Lee
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  5. W-J. Chen
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Correspondence to S. -C. Tung.

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Tung, S.C., Wang, PW., Huang, TL. et al. Bilateral adrenocortical adenomas causing ACTH-independent Cushing’s syndrome at different periods: A case report and discussion of corticosteroid replacement therapy following bilateral adrenalectomy. J Endocrinol Invest 27, 375–379 (2004). https://doi.org/10.1007/BF03351066

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