一名39岁的日本妇女表现出库欣综合征的典型临床症状,包括闭经和多毛症,2年。她最初的内分泌评估结果与双侧肾上腺肿块引起的ACTH非依赖性库欣综合征一致(左右直径3.1cm和2.4cm,分别)。血清脱氢表雄酮水平为6,901ng/mL(正常范围230-2,660ng/mL)。行双侧腹腔镜肾上腺切除术(先切除左肾上腺,3个月后切除右肾上腺)。左肾上腺切除术后,内分泌评估结果仍与ACTH非依赖性库欣综合征的诊断一致.血清硫酸脱氢表雄酮水平,然而,低于正常范围(143ng/mL)。出乎意料的是,患者的月经在左肾上腺切除术后2.5个月恢复。切除腺体的病理检查显示双侧肾上腺皮质腺瘤,一个在左边,直径为3厘米,右边两个直径分别为0.7厘米和1.3厘米。免疫组织化学分析显示侧链裂解,17α-羟化酶,3β-羟基类固醇脱氢酶,和每个腺瘤中的21-羟化酶免疫反应性。脱氢表雄酮-磺基转移酶免疫反应性在左腺瘤中明显,其中一个右侧腺瘤不明显,另一个右侧腺瘤较弱。这些结果与临床内分泌检查结果一致。我们是首例双侧皮质醇分泌腺瘤伴单侧硫酸脱氢表雄酮过度分泌的患者。纠正硫酸脱氢表雄酮过量后恢复月经,尽管皮质醇持续过量,表明肾上腺雄激素对调节月经周期的重要性。
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 ACTH-independent Cushing\'s syndrome due to bilateral adrenal masses (diameters 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. Dehydroepiandrosterone-sulfotransferase 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.