ACTH非依赖性肾上腺皮质大结节增生症的诊断与手术处理

Diagnosis and Treatment of ACTH Independent Macronodular Adrenal Hyperplasia

目的总结促肾上腺皮质激素(adrenocorticotrophic hormone,ACTH)非依赖性肾上腺皮质大结节增生症(ACTH independent macronodular adrenal hyperplasia,AIMAH)的诊治经验。方法回顾性分析5例AIMAH患者的临床资料。结果5例患者均具有库欣综合征的临床和生化特点,小剂量、大剂量地塞米松抑制试验均不被抑制,血浆ACTH水平低。4例患者行CT检查示双侧肾上腺大结节样增生改变,所有患者均经病理检查证实为双侧肾上腺大结节样或腺瘤样增生。例1、例2行单侧肾上腺切除后,分别于症状缓解3年和5年后复发,行对侧肾上腺全切除后治愈;例3分期行双侧肾上腺切除后治愈;例4、例5行单侧肾上腺切除后,前者5年症状持续缓解,后者仍在随访中。术后无Nelson综合征。结论AIMAH具有独特的内分泌、CT影像和病理学特点,是一种独立的库欣综合征病因,确诊主要依据病理检查。单侧肾上腺切除可获较长时间的症状缓解,应坚持随访患者的皮质醇水平和症状体征,如有复发可行对侧肾上腺切除。

Objective To investigate the diagnosis and treatment of ACTH independent macronodular adrenal hyperplasia （AIMAH）. Methods Five cases of AIMAH were retrospectively reviewed in terms of clinic features. Results All cases presented physical signs and biochemical evidence of Cushing＇s syndrome, High- and low-dose dexamethasone suppression tests failed to suppress cortisone secretion, showing unelevated ACTH levels in all cases. Bilateral massively enlarged adrenal glands were screened on CT scan in 4 cases, pathologically presented as macronodular or adenomatoid hyperplasia. Symptoms recurred in 2 cases after 3- and 5-year＇s remission postoperatively and were cured after the contralateral adrenalectomy. One case was cured by staging bilateral adrenalectomy. 1 case has relieved for 5 years and another was following up after unilateral adrenalectomy. There was no Nelson＇s syndrome in all cases. Conclusions AIMAH had unique endocrinological, radiological and pathological features, presenting as an independent etiological factor of Cushing＇s syndrome. Diagnosis of AIMAH mostly derived from pathological exam. Long-term remission can be achieved by unilateral adrenalectomy, contralateral adrenalectomy should be performed in case of recurrence when followed with periodical examination of symptom physical sign and serum concentration of cortisol.