胃肠道节细胞神经瘤6例临床病理分析  

Ganglioneuroma of gastrointestinal tract:a clinicopathological analysis of six cases

作　　者：何毅辉[1] 黄海建[1] 陈志忠[1] 江淑萍[1] 陈小岩[1] HE Yi-hui;HUANG Hai-jian;CHEN Zhi-zhong;JIANG Shu-ping;CHEN Xiao-yan(Department of Pathology,Fujian Provincal Hospital,Fuzhou350001,China)

机构地区：[1]福建省立医院病理科,福州350001

出　　处：《临床与实验病理学杂志》2020年第5期542-546,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨胃肠道节细胞神经瘤的临床病理学特征,以提高对其的认识水平。方法回顾性分析6例胃肠道节细胞神经瘤的临床病理学特征和免疫表型,并复习相关文献。结果6例患者中男性4例,女性2例,年龄43~72岁,平均55岁。肿瘤均位于结直肠,其中息肉样节细胞神经瘤4例,伴PTEN错构瘤综合征(PTEN hamartoma tumor syndrome,PHTS)的节细胞神经瘤性息肉病1例,伴神经纤维瘤病1型、神经内分泌瘤(neuroendocrine neoplasm,NET)和多发胃肠道间质瘤(gastrointestinal stromal tumors,GIST)的弥漫性节细胞神经瘤病1例。镜下见肿瘤由增生的Schwann细胞和散布其中的簇状、巢状排列的神经节细胞构成。Schwann细胞表达CD56、SOX-10、S-100蛋白,部分表达NSE、Syn、GFAP、NF,神经节细胞表达NSE、CD56、Syn、Calretinin、NeuN、S-100蛋白。结论节细胞神经瘤是胃肠道罕见的良性神经源性肿瘤,包含3个亚型:息肉样节细胞神经瘤、节细胞神经瘤性息肉病和弥漫性节细胞神经瘤病。各亚型具有独特的临床病理学特征,治疗应根据临床分型采取不同的处理方法,患者预后良好。Purpose To investigate the clinicopathological characteristics of gastrointestinal ganglioneuromas(GIGN),and to improve the understanding of the tumor.Methods The clinicopathological characteristics and immunophenotype of 6 cases of GIGN were retrospectively analyzed,and the relevant literatures were reviewed.Results Among the 6 patients,4 were male and 2 were female,aged from 43 to 72 years and the mean age was 55 years.The tumors were all located in the colorectum,including 4 cases of polypoid ganglioneuroma(PG),1 case of ganglioneuromatous polyposis(GP)with PTEN hamartoma tumor syndrome,and 1 case of diffuse ganglioneuromatosis(DG)with neurofibromatosis type 1,neuroendocrine tumor and multiple gastrointestinal stromal tumors.Histologically,tumors were composed of proliferating Schwann cells and clustered,nested ganglion cells.Immunohistochemically,Schwann cells expressed CD56,SOX-10,S-100 protein,some expressed NSE,Syn,GFAP and NF,ganglion cells expressed NSE,CD56,Syn,calretinin,NeuN,and S-100 protein.Conclusion GIGN is a rare benign neurogenic tumor of gastrointestinal tract,including three subtypes:PG,GP and GP.Each subtype has unique clinicopathological characteristics.Different treatment methods should be adopted according to different types.The prognosis is good.

关 键 词：胃肠道肿瘤 节细胞神经瘤 临床病理学特征 诊断 鉴别诊断 

分 类 号：R735.3[医药卫生—肿瘤]