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肾上腺外科疾病组织学分布及临床病理相关性研究

发布时间:2019-05-11 02:11
【摘要】: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%,醛固酮增多症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酮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。
[Abstract]:Objective to study the histologic distribution and epidemiological characteristics of adrenal surgical diseases diagnosed and treated in Beijing Union Medical College Hospital in the past 20 years. To explore the histologic characteristics of adrenal tumors and their correlation with clinical endocrine function. Methods 3334 patients with adrenal surgical diseases treated surgically from 1994 to 2013 were selected from 1994 to 2013, which could be verified by the medical record system of Beijing Union Medical College Hospital. To study the histologic distribution characteristics of adrenal surgical diseases and to understand the epidemiological characteristics of diseases from different histologic sources, such as age, sex, surgical mode and so on. 95 patients with adrenal diseases diagnosed and pathologically diagnosed by the Department of Urology of Beijing Union Medical College Hospital were randomly selected. Each patient was collected with a detailed medical history and pathological specimens were obtained. Immunohistochemical pathological staining was used to study the differentiation of adrenal tumor skin and medulla diseases. According to the characteristics of Endocrinology, 36 patients with adrenal diseases diagnosed and pathologically diagnosed by the Department of Urology of Beijing Union Medical College Hospital were randomly selected. The histologic distribution characteristics of cortical tumors and their correlation with endocrine function were further studied by histochemical pathological staining. (2) Immunohistochemical pathological staining was used to further understand the histologic distribution of cortical tumors and its correlation with endocrine function. All patients volunteered to participate in this study and signed informed consent forms for clinical specimens. Results A total of 3334 cases of adrenal surgical diseases admitted to Beijing Union Medical College Hospital from 1994 to 2013 were collected, which is the largest histologic distribution statistics of adrenal surgical diseases in China at present. A total of 17 types of cases were included, of which 1% of the diseases were adrenocortical adenoma, pheochromocytoma / paraganglioma, myeloid lipoma, metastases and ganglioneuroma according to the prevalence rate of adrenocortical adenoma, pheochromocytoma / paraganglioma. There were significant differences between different histologic distribution and age, disease, hospitalization days and surgical methods. In adrenocortical adenomas, 45.7% of nonfunctional adenomas, 39.5% of aldosteronism, 14.7% of cortisol hyperplasia and 0.2% of sex hormone abnormalities were found. The histologic distribution of men over 60 years old had its own characteristics. Adrenocortical cell carcinoma, metastasis and other relatively rare diseases should be consciously examined. It was previously thought that dysfunctional ganglioneuroma and myeloid lipoma might be complicated with clinical function. CgA,MelanA is a reliable immuno-histochemical marker for the differentiation of adrenal medulla and cortical tumors, but the differential effect of Melan-A on adrenocortical carcinoma is poor. CYP11B1,CYP11B2 and KCNJ5 can help to distinguish lesions with cortisol secretion. The specificity of KCNJ5 was 81%, and the negative predictive value of 11B2 was 92.2%. Conclusion the histologic distribution, age, sex, surgical mode and hospitalization days of adrenal surgical diseases are different. The main adrenal cortical tumors were nonfunctional adenoma, followed by aldosterone adenoma with globular zone secretion and Cushing adenoma with fasciculate zone in normal adrenal tissue, and pheochromocytoma was the main medulla-derived tumor. Immunohistochemical staining is easy to distinguish cortical tumors from medulla tumors, but it is difficult to distinguish histologically from cortical tumors. It can only be suggested from the state of endocrine function, and the source of cortical tumors can be located from the functional point of view. CYP11B1, Both CYP11B2 and KCNJ5 were highly expressed in cortisol adenomas.
【学位授予单位】:北京协和医学院
【学位级别】:博士
【学位授予年份】:2016
【分类号】:R736.6

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