肾上腺外副神经节瘤15例临床病理分析

Extra- adrenal paraganglioma:a clinicopathologic analysis of 15 cases

目的:探讨肾上腺外副神经节瘤的临床病理特征及鉴别诊断。方法:收集我科诊断的15例肾上腺外副神经节瘤患者的临床及病理资料进行分析,并复习相关文献。结果:15例肾上腺外副神经节瘤患者中男5例,女10例,年龄16~74岁。发病部位分别为后腹膜、椎管、颈部、膀胱、阴囊和阴道壁。组织学上肿瘤实质由排列成器官样或细胞球样结构的卵圆形或多边形主细胞和位于主细胞周围的梭形支持细胞所组成。15例副神经节瘤均诊断为良性。免疫组化显示肿瘤主细胞神经元特异性烯醇化酶、嗜铬素A、突触素阳性,而广谱细胞角蛋白、上皮膜抗原阴性;支持细胞表达S-100蛋白。结论:肾上腺外副神经节瘤发生部位多样,提高对不典型部位发生肿瘤的认识可减少误诊。

Objective:To explore the clinicopathologic features and differential diagnosis of extra-adrenal paraganglioma.Method:The clinical and histopathologic features of 15 cases of extra-adrenal paraganglioma confirmed by our department were analyzed and the related literatures was reviewed.Results:Among these 15 patients,5 were male and 10 were female aged from 16 to 74 years.The tumors were located in retroperitoneal,vertebral,neck,bladder,scrotum and vagina,respectively.Tumor were composed of oval or polygonal main cell and sustentacular cells which surrounded the main cell.The cells arranged in Zellballen or cellular.All cases were benign.Immunohistochemical investigations revealed main cells were positive for neuron specific endolase,chromogranin A,synaptophysin,but negative for cytokeratin,epithelial membrane antigen.Sustentacular cells were positive for S-100 protein.Conclusion:Extra-adrenal paraganglioma can arise in any location in adult tissues.Awareness of the existence of extra-adrenal paraganglioma in unusual locations will reduce errors in diagnosis.