节细胞性副神经节瘤临床病理学观察  

Gangliocytic paragangliomas:a clinicopathologic study

作　　者：马怡晖[1] 高汉青[1] 李文才[1] Ma Yihui;Gao Hanqing;Li Wencai(Department of Pathology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院病理科,450052

出　　处：《中华病理学杂志》2018年第12期941-944,共4页Chinese Journal of Pathology

摘　　要：目的探讨节细胞性副神经节瘤(gangliocytiCparaganglioma,GP)临床病理特征。方法收集郑州大学第一附属医院2011年1月至2017年12月诊断的4例GP患者资料。通过病历查询获得患者临床资料及病理诊断,采用HE染色及RocheBenchMarkXT全自动免疫组织化学检测仪检测广谱细胞角蛋白(CKpan)、突触素、嗜铬粒素A(CgA)、CD56、神经元特异性烯醇化酶(NSE)及神经丝蛋白(NF)等标志物。通过电话随访收集患者预后资料。结果4例患者中男性2例,女性2例,均以间断性腹痛、腹胀入院。发病年龄47~73岁,中位年龄56岁。术前均行CT检查显示十二指肠降段肠壁局限性增厚,并于增强后轻度强化;3例超声内镜检查示肿物为位于十二指肠降段黏膜或黏膜下层的低回声影。瘤体最大径0.6~1.8cm,平均1.2cm。镜下,肿瘤均由上皮样细胞、梭形细胞和节细胞样细胞构成,三者比例不一。上皮样细胞表达CKpan、突触素、CgA和CD56;梭形细胞表达S-100蛋白、SOX-10;节细胞样细胞表达NF、突触素、CgA和CD56;3种细胞均表达NSE。4例患者术后随访3~30个月,均未复发或转移。结论GP是一种好发于十二指肠的具有良性生物学特性的肿瘤,多可采用内镜下完整切除,预后极好。尽管其发生率极低,但对于发生在十二指肠,尤其是第二段位于黏膜及黏膜下层的病变,在鉴别诊断时应尽量考虑到GP的可能。Objective To investigate the clinicopathological features of gangliocytic paraganglioma (GP). Methods Clinical data and pathological diagnosis of the 4 cases of GP were obtained through the medical record inquiry from January 2011 to December 2017 at the First Affiliated Hospital of Zhengzhou University. Routine HE staining and immunohistochemistry of CKpan, Syn, CgA, CD56, NSE and NF were performed. Clinical follow-up of the patients was obtained through telephone communication. Results All 4 patients, including 2 male and 2 female patients, presented with intermittent abdominal pain and distention. The median age was 56 years. Preoperative CT showed local thickening of the duodenum wall with slight enhancement in all four cases. Endoscopic ultrasonography showed low level echo in the mucous layer and submucosa involved by the tumor in 3 of 4 cases. The maximal diameter of the tumor ranged from 0. 6 to 1. 8 cm with an average of 1. 2 cm. Microscopically, the tumors consisted of epithelioid, spindle and ganglion-like cells, and the proportion of the three cell types was different among cases. Epithelioid cells expressed CKpan, Syn, CgA and CD56. Spindle cells expressed S-100 protein and SOX-10 and ganglion-like cells expressed NF, Syn, CgA and CD56. All tumour cells expressed NSE. All 4 patients had no recurrence a post-surgery follow-up period of 3 to 30 months. Conclusions GP of the duodenum is a benign tumor with excellent prognosis after endoscopic excision. Although its incidence is very low, its diagnosis should be considered for any mass lesion of the duodenum, especially involving mucosa and submucosa of the second dudenal segment.

关 键 词：副神经节瘤 十二指肠 SOXE转录因子 

分 类 号：R730.264[医药卫生—肿瘤]