原发性色素性结节状肾上腺皮质病临床病理分析及文献复习  

Primary pigmented nodular adrenocortical disease:a clinicopathological analysis and review of literature

作　　者：周皓锬 陈东[1] 于玮[1] ZHOU Hao-tan;CHEN Dong;YU Wei(Department of Pathology,the Beijing Anzhen Hospital of Capital Medical University,Beijing 100029,China)

机构地区：[1]首都医科大学附属北京安贞医院病理科,北京100029

出　　处：《诊断病理学杂志》2024年第11期1046-1049,共4页Chinese Journal of Diagnostic Pathology

基　　金：首都医科大学附属北京安贞医院创新转化项目(编号:AZ2022-CXZH-10)。

摘　　要：目的探讨原发性色素性结节状肾上腺皮质病(PPNAD)的临床病理学特征。方法收集首都医科大学附属北京安贞医院2009年1月至2023年6月2例原发性色素性结节状肾上腺皮质病患者的临床资料、影像学检查、病理特征,进行HE染色及免疫组织化学染色,分析其组织形态及免疫组织化学表达特征,并进行相关文献复习。结果例1男,31岁,肿瘤发生在右侧肾上腺。例2女,63岁,肿瘤发生在左侧肾上腺。镜下观察肾上腺皮质均呈多结节状增生,增生的结节主要由较大的颗粒状嗜酸性粒细胞构成,胞质内含不等量脂褐素。免疫表型:Syn、CD56、α-Inhibin、SDHB呈阳性表达,NSE、CK部分呈阳性表达,CgA、S-100、EMA呈阴性表达,Ki-67增殖指数约为2%;病理诊断为原发性色素性结节状肾上腺皮质病。结论原发性色素性结节状肾上腺皮质病较为罕见,诊断时需综合影像学、组织病理学及免疫组织化学染色进行诊断,必要时辅助基因检测以明确诊断。Objective To investigate the clinicopathological characteristics of primary pigmented nodular adrenocortical disease(PPNAD).Methods Clinical data,imaging studies,and pathological features were collected from two PPNAD patients treated at Beijing Anzhen Hospital of Capital Medical University,from January 2009 to June 2023.Hematoxylin and eosin(HE)staining and immunohistochemical staining were performed to analyze the histomorphological and immunohistochemical characteristics.Relevant literature was reviewed.Results The first case involved a 31-year-old male with a tumor in the right adrenal gland.The second case involved a 63-year-old female with a tumor in the left adrenal gland.Microscopically,the adrenal cortex exhibited multinodular hyperplasia.The hyperplastic nodules were primarily composed of large granular eosinophilic cells containing varying amounts of lipofuscin in their cytoplasm.Immunophenotypically,the cells expressed Syn,CD56,α-Inhibin,and SDHB positively,while NSE and CK showed partially positive expression.CgA,S-100,and EMA were negatively expressed,with a Ki-67 proliferation index of approximately 2%.The pathological diagnosis was primary pigmented nodular adrenocortical disease.Conclusion PPNAD is rare and requires a comprehensive diagnosis combining imaging,histopathology,and immunohistochemical staining.Genetic testing may be necessary to confirm the diagnosis.

关 键 词：肾上腺 原发性色素性结节状肾上腺皮质病 临床病理特征 基因检测 

分 类 号：R736.6[医药卫生—肿瘤]