A case of Cushing’s syndrome due to bilateral cortisol-secreting adenomas with unilateral DHEAS oversecretion

Abstract

Cortisol-seCreting adrenal adenomas account for 10-15% of endogenous Cushing's syndrome (CS) cases [1]. Bilateral cortisol-secreting adenomas, however, are extremely rare [2][3][4][5][6]. In contrast to ACTHdependent CS and adrenocortical-carcinomas, adrenocortical adenomas generally (with rare exception [7]) only secrete glucocorticoids without co-secretion of adrenal androgens [8]. We herein report a rare case of CS due to bilateral cortisol-secreting adrenal adenomas (one on the left and two on the right) with unilateral (left-sided) dehydroepiandrosterone sulphate (DHEAS) oversecretion. This is a unique hormonal Abstract. A 39-year-old Japanese woman presented with typical clinical symptoms of Cushing's syndrome, including amenorrhea and hirsutism, for 2 years. The results of her initial endocrine evaluation were consistent with ACTHindependent Cushing's syndrome due to bilateral adrenal masses (diameter of 3.1 cm and 2.4 cm on the left and right, respectively). Serum dehydroepiandrosterone levels were 6,901 ng/mL (normal range 230-2,660 ng/mL). Bilateral laparoscopic adrenalectomies were performed (left adrenalectomy first and right adrenalectomy 3 months later). Following the left adrenalectomy, the results of the endocrine evaluations were still consistent with a diagnosis of ACTH-independent Cushing's syndrome. Serum dehydroepiandrosterone sulphate levels, however, were below the normal range (143 ng/mL). Unexpectedly, the patient's menstruation resumed 2.5 months after the left adrenalectomy. Pathological examination of the resected glands showed bilateral adrenocortical adenomas, one on the left with a diameter of 3 cm, and two on the right with diameters of 0.7 cm and 1.3 cm. Immunohistochemical analysis revealed side chain cleavage, 17α-hydroxylase, 3β-hydroxysteroid dehydrogenase, and 21-hydroxylase immunoreactivity in each adenoma. Dehydroepiandrosteronesulfotransferase immunoreactivity was pronounced in the left adenoma, less pronounced in one of the right adenoma and weak in the other right adenoma. These results were consistent with clinical endocrine findings. Ours is the first case of a patient with bilateral cortisol-secreting adenomas with unilateral oversecretion of dehydroepiandrosterone sulphate. Resumption of menstruation after the correction of the dehydroepiandrosterone-sulphate excess, despite persistent cortisol excess, indicates the importance of adrenal androgens for the regulation of the menstrual cycle.

ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

“…AVS was performed in eight cases to evaluate the hypersecretion of cortisol, and only two of them applied cortisol gradient adjusted by plasma aldosterone [ 9 , 10 ]. All patients underwent surgical resection of adenomas, including ten bilateral total adrenalectomy [ 6 , 11 – 19 ], three unilateral partial adrenalectomy with contralateral total adrenalectomy [ 9 , 10 , 20 ], one bilateral partial adrenalectomy [ 21 ] and one bilateral subtotal adrenalectomy [ 22 ]. All patients received glucocorticoid replacement therapy postoperatively.…”