Ayala A R, Basaria S, Udelsman R, Westra W H, Wand G S
Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
J Clin Endocrinol Metab. 2000 Aug;85(8):2903-6. doi: 10.1210/jcem.85.8.6749.
Although nonsecreting suprarenal embryonic remnants are frequently found in the urogenital tract, adenomatous transformation resulting in glucocorticoid excess is a rare phenomenon. We report a case of a 63-yr-old woman that presented with new-onset hirsutism, facial plethora, hypertension, centripetal obesity, and a proximal myopathy. The 24-h urinary free cortisol excretion rate was elevated, and the serum ACTH level was suppressed. The patient failed an overnight and low dose dexamethasone suppression test and did not respond to CRH stimulation. In light of the undetectable baseline morning ACTH levels and the blunt response to CRH, the diagnosis of corticotropin-independent Cushing's syndrome was made. Imaging studies revealed normal adrenal glands and enlargement of a left pararenal nodule incidentally observed 4 yr before the onset of symptoms. Dramatic resolution of symptoms was observed after surgical removal of the 3.5-cm mass. Pathological exam confirmed adrenocortical adenoma in ectopic adrenal tissue. The case reported here represents the unusual circumstance in which the development of adenomatous transformation of ectopic adrenal tissue has been prospectively observed with imaging studies. It illustrates the importance of considering ectopic corticosteroid-secreting tumors in the context of corticotropin-independent Cushing's syndrome.
尽管在泌尿生殖道中经常发现无分泌功能的肾上腺胚胎残余组织，但导致糖皮质激素过多的腺瘤样转化是一种罕见现象。我们报告一例63岁女性患者，出现新发多毛症、面部潮红、高血压、向心性肥胖和近端肌病。24小时尿游离皮质醇排泄率升高，血清促肾上腺皮质激素（ACTH）水平被抑制。该患者过夜及小剂量地塞米松抑制试验均未通过，对促肾上腺皮质激素释放激素（CRH）刺激无反应。鉴于基线晨ACTH水平检测不到且对CRH反应迟钝，诊断为促肾上腺皮质激素非依赖性库欣综合征。影像学检查显示肾上腺正常，在症状出现前4年偶然发现左肾旁结节增大。手术切除3.5 cm肿物后症状显著缓解。病理检查证实异位肾上腺组织中有肾上腺皮质腺瘤。本文报告的病例代表了一种不寻常的情况，即通过影像学研究前瞻性观察到异位肾上腺组织发生腺瘤样转化。它说明了在促肾上腺皮质激素非依赖性库欣综合征背景下考虑异位皮质类固醇分泌肿瘤的重要性。
