肠道节细胞神经瘤9例临床病理学分析  

Clinicopathological analysis of nine cases of ganglioneuromas of the intestine

作　　者：王锐[1] 李琴 姜勇[1] 吴立雪 WANG Rui;LI Qin;JIANG Yong;WU Lixue(Department of Pathology,West China Hospital,Sichuan University,Chengdu 610041,China;Chengdu Fifth People's Hospital,Chengdu 610041,China;Department of Pathology,West China Fourth Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院病理科,四川成都610041 [2]成都市第五人民医院,四川成都610041 [3]四川大学华西第四医院病理科,四川成都610041

出　　处：《西部医学》2024年第11期1606-1609,1614,共5页Medical Journal of West China

基　　金：四川大学华西医院学科卓越发展1·3·5工程临床研究孵化项目资助(2020HXFH024)。

摘　　要：目的探讨肠道节细胞神经瘤(GNs)的临床病理学特征。方法回顾性分析四川大学华西医院病理科2008年9月—2021年9月经病理诊断为肠道GNs病例的临床病理学特征并复习相关文献。结果9例肠道GNs患者,男性5例,女性4例,中位年龄53岁(21~64岁)。其中息肉样节细胞神经瘤(PG)6例,节细胞神经瘤性息肉病(GP)1例伴管状腺瘤,弥漫性节细胞神经瘤病(DG)2例,其中1例伴神经鞘瘤。镜下GNs由神经节细胞和梭形Schwann细胞构成,免疫组织化学神经节细胞表达神经元特异性烯醇化酶(NSE)、突触素(Syn)、S-100蛋白、神经元特异核蛋白(NeuN)、神经丝蛋白(NF);梭形Schwann细胞表达S-100蛋白、神经丝蛋白(NF);Ki-67增殖指数<1%。9例肠道GNs均未合并遗传性综合征。结论肠道GNs是一种分化成熟的良性神经源性肿瘤,可分为3个亚型:PG、GP及DG,各亚型临床病理特征略有不同,预后较好,但应密切随访。Objective To investigate the clinicopathologic features of intestinal ganglioneuromas(GNs).Methods To retrospectively analyze the clinicopathological features of the cases diagnosed as intestinal GNs by pathology in the Department of Pathology,West China Hospital,Sichuan University,from September 2008 to September 2021 and review the relevant literature.Results Nine patients with intestinal GNs,five males and four females,with a median age of 53 years(21~64 years).There were 6 cases of polypoid ganglioneuroma(PG),1 case of ganglioneuromatous polyposis(GP)with tubular adenoma,2 cases of diffuse ganglioneuromatosis(DG),and 2 cases of diffuse ganglioneuromatosis(DG),1 of which was associated with a Neurosheath tumor.Microscopically,ganglioneuromas were composed of ganglion cells and spindle-shaped Schwann cells.Immunohistochemically,ganglion cells expressed neuron-specific enolase(NSE),synaptophysin(Syn),S-100 protein,neuron-specific nuclear protein(NeuN),and neurofilament protein(NF);spindle Schwann cells express S-100 protein and neurofilament protein(NF);and Ki-67 proliferation index was<1%.9 cases of intestinal GNs were not combined with hereditary syndromes.Conclusion Intestinal GNs are maturely differentiated benign neurogenic tumors,which can be divided into three subtypes:PG,GP and DG.Each subtype has slightly different clinicopathological features and has a better prognosis,but should be closely followed up.

关 键 词：肠道 节细胞神经瘤 临床病理分析 遗传性综合征 

分 类 号：R735.3[医药卫生—肿瘤]