肾上腺髓质增生症七例诊治分析  

Diagnosis and treatment of adrenal medullary hyperplasia

作　　者：黄钟明[1] 李汉忠[1] 纪志刚[1] 毛全宗[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院泌尿外科,100730

出　　处：《中华医学杂志》2014年第18期1413-1415,共3页National Medical Journal of China

摘　　要：目的探讨肾上腺髓质增生症（AMH）的临床诊治特点。方法总结1996年1月至2013年5月北京协和医院泌尿外科收治的7例AMH患者完整的临床资料进行分析。结果7例患者中男3例，女4例，年龄23～69岁，中位年龄36岁。患者均因高血压就诊，其中2例有发作性血压升高。CT均提示单侧肾上腺结节，左侧5例，右侧2例。2例患者有血钾低于正常，内分泌检查提示2例24h尿儿茶酚胺升高，1例24h尿游离皮质醇升高同时促肾上腺皮质激素（ACTH）降低，1例有立位肾素活性降低。3例术前行^131I-间位碘代苄胍（^131I-MIBG）显像检查1例阳性。术前疑诊为嗜铬细胞瘤2例，肾上腺皮质腺瘤3例（醛固酮腺瘤2例，库欣腺瘤1例），肾上腺不明结节2例。7例患者均进行手术治疗，4例行一侧肾上腺切除，3例行肿物切除。术后病理均为肾上腺髓质增生，合并有库欣腺瘤或肾上腺皮质增生各1例。术后1例患者血压完全恢复正常，其他患者血压较术前均有不同程度下降，仍高于正常。结论肾上腺髓质增生术前诊断困难，高血压是其共同特征，CT有一定的诊断意义，确诊依赖于病理学检查；术前及术中难以明确诊断，手术范围不彻底导致治疗效果不理想。Objective To explore the clinical characters of adrenal medullary hyperplasia （AMH）. Methods The clinical data of 7 cases of AMH admitted from 1996 to 2013 were analyzed with a review of literature. Results There were 3 males and 4 females with a median age of 36 （23 - 69） years. All had hypertension at first diagnosis and 2 were paroxysmal. Computed tomography （CT） scan revealed unilateral adrenal nodule in all cases, including left （ n = 5） and right （ n = 2） side. The diameter of nodules was 0. 9 cm to 3.0 cm. Hypokalemia was found in 2 cases. And 24-hour output of urine catecholamine was higher than normal level in 2 cases. An abnormal rise of 24-hour output of urine free cortisol was found in 1 case with a lower level of adrenocorticotropic hormone （ACTH） simultaneously while a lower level of plasma renin activity in vertical position was found in another case. Three patients received ^131I-meta-iodobenzylgnanidine scan and one had positive result. Pre-operative diagnosis included pheochromocytoma （ n = 2）, adrenocortical adenoma （ n = 3 ） （2 aldosterone-producing adenomas, 1 Cushing adenoma） and undetermined adrenal tumor （ n = 2）. All underwent surgery, including unilateral adrenalectomy （ n = 4 ） and resection of adrenal disease （ n = 3 ）. All cases had a pathologic diagnosis of AMH. One had concurrent Cushing adenoma while another with cortical hyperplasia. One case achieved normotension postoperatively while others showed varying decreases of blood pressure, but remained hypertensive. Conclusion With a common feature of hypertension, AMH is difficult to diagnose preoperatively. CT scan has a certain diagnostic significance and a definite diagnosis depends on pathological examination; because of inaccurate preoperative and intraoperative diagnosis, the extent of surgery may be incomplete and results in unsatisfied outcomes.

关 键 词：肾上腺髓质 诊断 治疗 

分 类 号：R736.6[医药卫生—肿瘤]