ACTH非依赖性大结节样肾上腺增生症7例报告并文献复习

Adrenocorticotropic hormone-independent macronodular adrenocortical hyperplasia-report of seven cases report and review of literature

目的探讨ACTH非依赖性大结节样肾上腺增生(AIMAH)的临床及病理特点,提高对AIMAH诊断和治疗的认识。方法分析1997～2005年收治的7例AIMAH的临床和实验室资料。结果7例患者中5例有典型库欣综合征(CS)的临床表现,包括满月脸,向心性肥胖,高血压等;2例以高血压等非特异性症状就诊。部分患者有低血钾(43%)和继发性糖尿病(71%)。实验室检查血浆ACTH水平低下,皮质醇水平增高或节律紊乱,6例小剂量、大剂量地塞米松抑制试验均不被抑制。CT检查示双侧肾上腺明显增大,呈结节样改变。2例行单侧肾上腺全切术,4例行双侧切除术,1例在等待手术期间因脑出血死亡。术后5例行糖皮质激素替代治疗。病理示肿瘤多呈黄色或灰黄色弥漫性结节样改变,直径0.3～7.0cm。6例术后随诊至今未复发,未发生Nelson综合征。结论AIMAH是库欣综合征独特的亚型,有其特有的内分泌、影像及病理改变。治疗以双侧肾上腺切除、分期双侧肾上腺切除或单侧切除为主。术后辅以激素替代治疗。

Objective To study the characteristics of clinical and laboratory findings in patients with adrenocorticotropic hormone （ACTH） independent macronodular adrenocortical hyperplasia （AIMAH）. Methods The clinical and laboratory findings of 7 cases of AIMAH admitted between 1997 and 2005 were reviewed retrospectively. Results 5 of the 7 patients showed typical clinical features of Cushing＇s syndrome, including moon face, central obesity, hypertension, etc. A part of patients had hypokalemia （43%） and secondary diabetes mellitus （71%）. There was lowering of plasma ACTH levels, and an increased cortisol secretion or disturbance in or circadian rhythm. In 6 patients, a low dose or high dose of dexamethasone suppression tests failed to suppress cortisol secretion. CT scan showed bilateral macronodular adrenal hyperplasia. Pituitary MR imaging for pituitary was negative in 6 patients. Unilateral total adrenalectomy was performed in 2 cases. 4 cases were treated by bilateral adrenalectomy. One patient died of stroke before operation. 5 cases received glucocorticoid re- placement therapy after operation. Pathologic examination showed diffuse yellow or brown nodules measuring 0.3 cm-7.0 cm in diameter in all the specimems of 6 patients who undement operation. There was no recurrence in all the patients ofter surgery during the follow-up period. No Nelson syndrome occurred in these 6 patients postoperatively. Conclusion AIMAH is a rare cause of Cushing＇s syndrome with unique clinical, CT image and pathological findings. Bilateral adrenalectomy in one stage or in stages, or unlateral adremectomy is indicated on the basis of clinical and CT findings.