背景:在原发性醛固酮增多症(PA)的背景下报道了越来越多的醛固酮和皮质醇产生腺瘤(A/CPAs)病例。这些患者中大多数患有PA并发亚临床库欣综合征;明显库欣综合征(CS)并发醛固酮增多症的病例报道较少。然而,右肾上腺存在共分泌性肿瘤,一个分泌皮质醇的腺瘤和一个产生醛固酮的结节(APN)存在于左肾上腺,和醛固酮产生微结节(APM)存在于两个肾上腺,尚未报告。这里,我们报告了这样一个案例,提供了深刻的见解,这种疾病的临床和病理特征的多样性。
方法:患者为四川大学华西医院肾上腺疾病诊疗中心45岁女性。病人出现高血压,月亮形的脸,中心性肥胖,脂肪堆积在脖子后面,皮质醇昼夜节律消失,ACTH<5ng/L,地塞米松抑制皮质醇升高失败,直立醛固酮/肾素活性>30(ng/dL)/(ng/mL/h),生理盐水输注后血浆醛固酮浓度>10ng/dL。根据上述情况,她被诊断为非ACTH依赖性CS合并PA.肾上腺静脉采样显示双侧肾上腺皮质醇和醛固酮分泌无偏侧化。机器人辅助腹腔镜切除左侧肾上腺皮质腺瘤。然而,高血压,手术后疲劳和体重增加没有缓解;此外,小腹出现紫色条纹,腹股沟区域和大腿内侧,伴有全身关节痛。一个月后,右侧肾上腺皮质腺瘤也被切除.CYP11B1在双侧肾上腺皮质腺瘤中表达,CYP11B2也在右肾上腺皮质腺瘤中表达。APN存在于左肾上腺,APM存在于双侧肾上腺皮质腺瘤附近的肾上腺皮质。又一次手术后,她的血清皮质醇和血浆醛固酮恢复到正常范围,除了略高的ACTH。
结论:此案例表明有必要评估PA的存在,即使在CS有明显的症状。由于CS和PA患者可能有更复杂的肾上腺病变,诊断需要更多数据。
BACKGROUND: There is an increasing number of cases of aldosterone- and cortisol-producing adenomas (A/CPAs) reported in the context of primary aldosteronism (PA). Most of these patients have PA complicated with subclinical Cushing\'s syndrome; cases of apparent Cushing\'s syndrome (CS) complicated with aldosteronism are less reported. However, Co-secretory tumors were present in the right adrenal gland, a cortisol-secreting adenoma and an aldosterone-producing nodule (APN) were present in the left adrenal gland, and aldosterone-producing micronodules (APMs) were present in both adrenal glands, which has not been reported. Here, we report such a
case, offering profound insight into the diversity of clinical and pathological features of this disease.
METHODS: The
case was a 45-year-old female from the adrenal disease diagnosis and treatment centre in West China Hospital of Sichuan University. The patient presented with hypertension, moon-shaped face, central obesity, fat accumulation on the back of the neck, disappearance of cortisol circadian rhythm, ACTH < 5 ng/L, failed elevated cortisol inhibition by dexamethasone, orthostatic aldosterone/renin activity > 30 (ng/dL)/(ng/mL/h), and plasma aldosterone concentration > 10 ng/dL after saline infusion testing. Based on the above, she was diagnosed with non-ACTH-dependent CS complicated with PA. Adrenal vein sampling showed no lateralization for cortisol and aldosterone secretion in the bilateral adrenal glands. The left adrenocortical adenoma was removed by robot-assisted laparoscopic resection. However, hypertension, fatigue and weight gain were not alleviated after surgery; additionally, purple striae appeared in the lower abdomen, groin area and inner thigh, accompanied by systemic joint pain. One month later, the right adrenocortical adenoma was also removed. CYP11B1 were expressed in the bilateral adrenocortical adenomas, and CYP11B2 was also expressed in the right adrenocortical adenomas. APN existed in the left adrenal gland and APMs in the adrenal cortex adjacent to bilateral adrenocortical adenomas. After another surgery, her serum cortisol and plasma aldosterone returned to normal ranges, except for slightly higher ACTH.
CONCLUSIONS: This
case suggests that it is necessary to assess the presence of PA, even in CS with apparent symptoms. As patients with CS and PA may have more complicated adrenal lesions, more data are required for diagnosis.