异位肾上腺皮质腺瘤——罕见的皮质醇增多症病因

Ectopic adreocortical adenoma——An uncommon etiology of Cushing′s syndrome

本文主要报道1例由异位肾上腺皮质腺瘤导致的皮质醇增多症。该患者为37岁女性,因"发现血压升高2年,双下肢乏力2个月"就诊,体格检查:血压160/116 mmHg(1 mmHg=0.133 kPa),体重指数27.47 kg/m^2,满月脸,颈背部脂肪厚,腹部皮肤见紫纹,双下肢Ⅱ度水肿。实验室检查提示皮质醇分泌增多,失去正常的昼夜节律性,促肾上腺皮质激素(adrenocorticotropic hormone, ACTH)始终处于抑制状态,小剂量地塞米松不能抑制皮质醇分泌。肾上腺计算机断层扫描(CT)提示右侧后腹膜占位,压迫右侧肾门,两侧肾上腺形态纤细,未见明显腺瘤及增生。诊断为非ACTH依赖性皮质醇增多症。经腹腔镜下肿块切除后临床症状好转。术后病理提示异位肾上腺皮质腺瘤。因此,当非ACTH依赖的皮质醇增多症诊断明确,而影像学检查提示双侧肾上腺未见异常或萎缩时,应考虑是否存在异位肾上腺增生或腺瘤,需要逐一排查,发现病灶进行正确的治疗。

We report a case of cushing′s syndrome caused by ectopic adreocortical adenoma. The patient is a 37 years old woman, she was admitted to our hospital for " 2 years history of hypertension and weakness in both lower extremities for 2 months" . Physical examination revealed: blood pressure 160/116 mmHg(1 mmHg=0.133 kPa), body mass index 27.47 kg/m^2, moon-face, increased fat in the neck and back, purple marks on abdominal skin, with Ⅱ degree edema of both lower extremities. Laboratory examination revealed that serum cortisol levels were elevated, loss of normal circadian rhythm, and serum adrenocorticotropic hormone (ACTH) was suppressed, the level of cortisol could not be suppressed in low dose desamethasone suppression test. Adrenal computed tomography (CT) revealed a nodule in the right retroperitoneum, compression of the renal hilum, no bilateral adrenal adenoma and hyperplasia were found. This patient was diagnosed as corticotropin-independent Cushing′s syndrome unequivocally. The clinical symptoms were relieved after successful laparoscopic retroperitoneum resection of the nodule. Pathological exam confirmed adrenocortical adenoma in ectopic adrenal tissue. Thus, we should consider the ectopic corticosteroid-secreting tumor in the context of corticotropin-independent Cushing′s syndrome, especially when the imaging studies of adrenal revealed bilateral adrenal glands were normal or atrophic, which helped to make an appropriate strategy treatment.