目的：研究北京协和医院20年间诊治的肾上腺外科疾病的组织学分布及流行病学 特点。探寻肾上腺肿瘤组织学特点，探究组织病理学免疫标记物及其与临床内分 泌功能的相关性。 方法：收录北京协和医院北京协和医院病案系统可查证的1994年至2013年期间 收治为行手术治疗的肾上腺外科疾病患者，筛选病理资料完整的患者3334例，研 究肾上腺外科疾病组织学分布特点，了解不同组织学来源疾病的年龄、性别、手 术方式等流行病学特点。随机抽取北京协和医院泌尿外科确诊并明确病理诊断的 肾上腺疾病患者95例，对每例患者进行详实的病史采集并获取病理标本。采用免 疫组织化学病理染色研究肾上腺肿瘤皮、髓质疾病的鉴别。依据内分泌学特点随 机抽取北京协和医院泌尿外科确诊并明确病理诊断的肾上腺疾病患者36例，采用 免疫组织化学病理染色研究进一步了解皮质肿瘤组织学分布特点及与内分泌功能 的相关性。所有患者均自愿参与本研究，并签署临床标本知情同意书。 结果：本研究共收集北京协和医院1994年至2013年这20年间收治的3334例病 历资料完整肾上腺外科疾病病例，是目前国内最大规模的肾上腺外科疾病组织学 分布统计。共包含17类病例，其中单病种疾病 >1%的疾病依照患病率依次为：肾 上腺皮质腺瘤、嗜铬细胞瘤/副神经节瘤、髓样脂肪瘤、转移癌和节细胞神经瘤。 不同组织学分布与年龄、疾病、住院天数、手术方式存在显著差异。肾上腺皮质 腺瘤中无功能腺瘤45.7%，醛固醐增多症39.5%，皮质醇增多症14.7%，性激素异 常0.2%。 60岁以上男性组织学分布具有自身特点，应有意识排查肾上腺皮质细 胞癌、转移癌等相对少见的疾病。既往认为无功能的节细胞神经瘤和髓样脂肪瘤 可能合并临床功能。皮髓质肿瘤的鉴别方面，CgA、 MelanA为可靠的肾上腺髓质和 皮质鉴别的免疫组化标记物，但Melan-A对肾上腺皮质癌的鉴别效力差CYP11B1、 CYP11B2和KCNJ5可帮助鉴别具有皮质醇分泌功能的病灶，其中KCNJ5的特异性高 达81%， CYP11B2的阴性预测值可达92.2%。 结论：肾上腺外科疾病的组织学分布不同，年龄、性别、手术方式和住院天数表现 各异。肾上腺皮质肿瘤以无功能腺瘤为主，其次为正常肾上腺组织中以球状带分泌 为主的醛固醐腺瘤及束状带的库欣腺瘤为主，髓质来源肿瘤以嗜铬细胞瘤为主。免 疫组织化学染色较易鉴别皮、髓质肿瘤，但对于皮质来源肿瘤的组织学区分困难， 仅能从内分泌功能状态有一定提示，从功能学角度定位皮质肿瘤来源。CYP11B1、 CYP11B2和KCNJ5均在皮质醇腺瘤中高表达。

Objective: Histology distribution of adrenal surgical disease at Peking Union Medical College Hospital recent 20 years.The pathological feature and differential diagnosis of adrenal tumor and the function of adrenocortical tumor. Method: Set the inclusion and exclusion criteria, collect the clinical data of adrenal surgical disease who receive surgery in PUMC hospital between Jan 1994 to Dec 2013. The analyze adrenal surgical disease clinical date. Immunohistochemically was used to detect the expression of CgA, Melan-A,CYP11B1 and CYP11B2. Analysis the results using SPSS 22.0 software. Result : This research was the largest cases reports on adrenal surgical disease, which including 3334 cases and 17 different diseases. The most common disease was adrenal cortical adenoma , pheochromocytoma/paraganglioma, myelolipoma, adrenal metastasis and ganglioneuroma There are significance differences between the histopathology kinds. CgA and Melan-A could be considered as reliable immunohistochemical marks to differential diagnosis the adrenal masses from adrenal cortical or adrenal medulla.CYP11B1, CYP11B12 and KCNJ5 show a high expression on adrenal cortical-producing lesion, which can be used to diagnosis the adrenal cortical-producing lesions from others. Conclusion: Adrenal surgical disease shows tremendous difference in age, gender, surgery and hospital stay in each kind histological disease. Non-function disease was the most common type in adrenal cortical adenoma, and then were adrenal aldosterone-producing adenoma and cortical-producing adenoma. Immunohistochemical marks could differential diagnosis the adrenal masses from adrenal cortical or adrenal medulla. CYP11B1, CYP11B12 and KCNJ5 show a high expression on adrenal cortical-producing leison.It was the first time to show the expression of KCNJ5 in cortical-producing lesion. Nonetheless, our study have some limits, the statistics difference could not equal to clinical practicability and the sample size of the immunohistochemical research are small, which means our conclusion need the father research to supported.