Objective To investigate the clinical features, diagnosis and treatment of adrenocorticotropic hormone (ACTH)-independent bilateral adrenocortical hyperplasias (BAHs). Methods The clinical data of 21 cases of ACTH-inde-pendent BAHs treated during Jan. 1980 to May 2011 were retrospectively analyzed. The 21 cases, including 6 males and 15 females, with a mean age of 34.3(ranging 12~58) years, consisted of 8 ACTH-independent macronodular adrenal hyperplasia (AIMAH) cases and 13 primary pigmented nodular adrenocortical disease (PPNAD) cases. 18 patients demonstrated typical Cushing's syndrome (CS) and 3 presented weight gain or hypertension without any signs of CS. CT scan showed massive bilateral adrenal enlargement containing multiple macronodules in 8 patients, bilateral adrenal glands slightly thickening or small multinodular adrenal glands in 6 patients, unilateral adrenal nodule in 2 patients, and the other 5 cases were of "normal" size. 16 patients received unilateral adrenalectomy, 3 bilateral adrenalectomy, and 2 bilateral subtotal adrenalectomy. Results Pathological examination showed that 8 cases were AIMAH and 13 were PPNAD. During the follow-up of 16 to 120 months, the clinical symptoms of CS disappeared in all cases. The 16 patients with unilateral adrenalectomy had urinary free cortisol and serum cortisol within normal range and no sign indicating further enlargement of the residual adrenal glands. The 3 patients with bilateral adrenalectomy were cured and received glucocorticoid replacement, and Nelson's syndrome was not observed. Conclusion AIMAH and PPNAD are rare subtypes of ACTH-independent CS. Unilateral adrenalectomy may be an effective and safe treatment for selected patients with AIMAH and PPNAD. It may achieve long-term remission of CS syndrome. A second surgery to remove the contralateral gland may be needed if CS recurs during follow-up, and lifelong treatment with glucocorticoid should be given subsequently.%目的 探讨促肾上腺皮质激素(adrenocorticotropic hormone,ACTH)非依赖性双侧肾上腺皮质增生的临床特点与诊治经验.方法 回顾性分析1980年1月至2011年5月诊治21例ACTH非依赖性双侧肾上腺皮质增生患者资料,包括8例ACTH非依赖性肾上腺皮质大结节样增生(AIMAH)和13例原发性色素性肾上腺结节增生(PPNAD).男6例,女15例.年龄12～58岁,平均34.3岁.临床表现为典型库欣综合征(Cushing's syndrome,CS) 者18例,表现为体重增加、高血压等非特异性症状者3例.CT检查提示双侧肾上腺结节样增生改变者14例,2例提示左肾上腺占位;5例肾上腺未见明显异常.双侧肾上腺全切3例;双侧肾上腺次全切除术2例,为初次手术行单侧肾上腺切除,术后库欣症状复发,再次行对侧肾上腺次全切除术;单侧肾上腺切除16例.结果 手术标本病理结果报告8例为AIMAH,13例为PPNAD.随访16～120个月,CS症状消失.16例单侧肾上腺切除术后血尿皮质醇、血钾、血糖等均恢复正常,对侧肾上腺无明显增大;3例行双侧肾上腺全切者术后醋酸可的松终身替代治疗,无尼尔森综合征发生;2例肾上腺次全切除者系一侧肾上腺全切术后随访中库欣症状复发,行对侧肾上腺次全切术后症状完全缓解.结论 AIMAH和PPNAD均为库欣综合征中罕见的特殊类型,首次手术可行单侧肾上腺切除术缓解症状.术后应密切随访,对症状不缓解或复发者可行对侧肾上腺切除或次全切除.
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