胃肠道炎症性纤维性息肉37例临床病理学观察  

Inflammatory fibroid polyp of the gastrointestinal tract： a clinicopathologic features of 37 cases

作　　者：刘丹[1] 王坚[2] 陈森 肖芹[1] 朱长仁[1] 蒋记心[1] 王翠梅[1] 顾学文[1] 

机构地区：[1]江苏省苏北人民医院病理科,扬州225001 [2]复旦大学附属肿瘤医院病理科复旦大学上海医学院肿瘤学系,200032 [3]江苏省康复医疗集团病理中心

出　　处：《中华病理学杂志》2016年第6期381-386,共6页Chinese Journal of Pathology

基　　金：国家自然科学青年基金（81402936）;江苏省苏北人民医院院级扶持新技术项目（fcjs201410）

摘　　要：目的探讨胃肠道炎症性纤维性息肉（IFP）的临床病理学特征、发病机制、诊断及鉴别诊断。方法回顾性分析37例IFP患者的临床、病理资料及免疫表型特点，进行KIT基因第9、11、13、17号外显子检测，血小板源生长因子受体仅（PDGFRA）基因第12、18号外显子检测，并复习相关文献。结果患者男性9例，女性28例。年龄37—78岁，平均57岁。肿瘤部位：22例位于胃窦，9例位于回肠，3例位于贲门，1例位于胃角，1例位于胃体，1例位于十二指肠。肿瘤最大径0．5～5．5CITI，平均3．0cm。大体观察呈息肉状隆起型病变，部分伴黏膜溃疡形成。镜下观察胃IFP由梭形细胞围绕小血管及黏膜腺体形成经典的“洋葱皮样”结构为特征，部分区呈交织状或束状排列；小肠IFP大部分病例缺乏“洋葱皮样”结构，组织疏松水肿，由星形或上皮样细胞构成，具有突出的毛细血管网，瘤细胞均匀分布于疏松水肿样间质内；2种组织学形态均伴有较多以嗜酸性粒细胞为主的炎性细胞浸润。瘤组织多位于黏膜及黏膜下层，其中1例小肠IFP向下生长累及肠壁浅肌层。免疫组织化学标记显示，37例均表达波形蛋白、CD34，18例表达PDGFRA。分子病理检测KIT基因第9、11、13、17号外显子及PDGFRA基因第12、18号外显子，结果显示均无KIT基因突变，其中4例IFP存在PDGFRA基因第18号外显子突变，突变形式为D842V，5例伴有第12号外显子突变，导致P．566．571delSPDGHEinsR。30例获得随访，无复发及转移。结论胃肠道IFP主要呈现2种组织学形态，且存在PDGFRA基因激活突变，是消化道良性间叶源性肿瘤，而非炎性反应性病变。Objective To study the clinicopathologic features, pathogenesis, and differential diagnosis of inflammatory fibroid polyp （ IFP ） of the gastrointestinal tract. Methods The clinical and pathologic findings of 37 IFPs in the gastrointestinal tract were retrospectively analyzed. Immunohistochemical study and KIT, PDGFRA molecular analysis were carried out and the literatures reviewed. Results There were 9 males and 28 females. The median age was 57 （ range 37 to 78 ） years. Twenty-two were in the antrum, nine in the ileum, three in the cardia, and one each in the gastric angle, corpora ventrieuli and duodenum. The lesion ranged in size from 0. 5 to 5.5 cm（ mean 3 cm）. Grossly, the majority appeared as a solitary non-encapsulated, submucosal, polypoid lesion. There was associated mueosal ulceration in three cases. Microscopically, the gastric lesions showed spindle-shaped cells arranged in an onion skin-like pattern around vessels and mucosal glands in a concentric formation. But the lesions in the ileum represented Vanek＇s tumor subtype devoid of concentric formations, with spindled to epithelioid ceils dispersed in edematous stroma. Most of the lesions were in the mucosa and submucoma, but one small intestinal IFP infiltrated the muscularis propria. The inflammatory component of the lesions consisted predominantly of lymphocytes and eosinophils. Immunohistochemically, all cases displayed diffuse reactivity for vimentin and CD34; and 18 expressed PDGFRA. Analysis of KIT and PDGFRA mutations was performed in 18 cases. No KIT mutations were identified. However, four cases harbored activating mutations in PDGFRA exon 18 （D842V）, five showed mutations in exon 12 （p. 566-571delSPDGHEinsR）. Follow-up in 30 cases showed no recurrence or metastasis. Conclusions IFPs not only exhibit two morphologies, but also show mutations in the PDGFRA gene. IFP is a benign mesenchymal tumor rather than a reactive lesion.

关 键 词：胃肠疾病 DNA突变分析 受体 血小板源生长因子α 免疫组织化学 诊断 鉴别 

分 类 号：R57[医药卫生—消化系统]