促肾上腺皮质激素非依赖性肾上腺皮质大结节样增生的外科治疗

Surgical treatment of adrenocorticotropin-independent macronodular adrenal hyperplasia

目的探讨促肾上腺皮质激素（adrenocnrticotropic hormone，ACTH）非依赖性肾上腺皮质大结节样增生（ACTH independent macronodul aradrenal hyperplasia，AIMAH）的诊治经验。方法回顾性分析1972年8月至2010年7月诊治14例AIMAH患者资料。男5例，女9例。年龄26～58岁，平均45岁。临床表现为典型库欣综合征（Cushing syndrome，CS）者10例，表现为体质量增加、高血压或高血糖等非特异性症状者4例。生化检查示皮质醇分泌昼夜节律消失，大、小剂量地塞米松抑制试验均’不被抑制。CT检查示双侧肾上腺弥漫性增大伴多发结节。14例均行开放手术治疗，其中单侧肾上腺切除5例；一侧肾上腺全切，对侧肾上腺次全切除6例；双侧肾上腺同时切除3例。结果14例病理诊断均为肾上腺大结节样或腺瘤样增生。随访12～120个月，平均69个月，CS症状消失。5例单侧肾上腺切除者术后血、尿皮质醇值恢复正常，对侧肾上腺无明显增大。3例双侧。肾上腺切除患者中，1例术后7d发生肾上腺皮质功能危象而死亡，2例术后出现皮质功能减退症状，给予糖皮质激素替代治疗。其他患者病情稳定，无Nelson综合征出现。结论AIMAH具有独特的临床症状特点，是cs的一种独立罕见类型。首次手术多行单侧肾上腺切除术，可获较长时间的症状缓解。术后应密切随访患者的皮质醇水平、症状和体征，对症状不缓解或复发者可行对侧肾上腺切除或次全切除。

Objective To investigate the diagnosis and treatment of adrenocortieotropin-independent macronodular adrenal hyperplasia （AIMAH）. Methods The clinical data of 14 cases of AIMAH from August 1972 to July 2010 were retrospectively analyzed. The cases included 5 males and 9 females with a mean age of 45 （range 26 to 58） years. Ten patients demonstrated typical Cushing＇s syndrome （C$） and 4 patients presented with weight gain, hypertension or diabetes mellitus without any signs of CS. The circadian rhythm of serum cortisol was abnormal. Low and high dose dexamethasone suppression tests failed to suppress cortisol secretion. CT scan showed bilateral enlargement of the adrenal glands with multiple ma- cronodules. All patients underwent open surgery, including 5 cases of unilateral adrenalectomy, 6 cases of adrenalectomy combined with contralateral subtotal adrenaleetomy and 3 cases of bilateral adrenaleetomy. Results It was established by pathological examination that all patients had bilateral adrenal macronodular or adenomatoid hyperplasia. During the mean follow-up of 69 months （range 12 to 120 months） , the clinical symptoms of CS disappeared after surgery in all cases. The 5 patients who received unilateral adrenalectomy had urinary free cortisol and serum cortisol within normal ranges and no further enlargement of the contralateral gland was noticed. Among the 3 patients who received bilateral adrenalectomy, 1 case died of adrenalcrisis on day seven post-operation. The remaining 2 cases presented with adrenal insufficiency but returned to normal after glucocortieoid replacement therapy. Nelson＇s syndrome was not observed in the other patients. Conclusions AIMAH has unique endocrinological and pathological features, presenting as an independent etiology of CS. Unilateral adrenaleetomy appears to be an effective and safe alternative treatment for AIMAH and long-term remission can be achieved. Bilateral adrenalectomy or unilateral adrenaleetomy combined with contralateral subtotal adrenalectomy may be performed if the symptoms have not improved or recurred after unilateral adrenalectomy.