胃肠道及系膜反应性结节状纤维性假瘤临床、内镜及病理特征分析
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1.南京大学医学院附属鼓楼医院病理科;2.芜湖市第五人民医院病理科;3.南京大学医学院附属鼓楼医院消化内科

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Clinical, endoscopic and pathological features of gastrointestinal and mesenteric reactive nodular fibrous pseudotumor
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Department of Pathology,Nanjing Drum Tower Hospital,the Affiliated Hospital of Nanjing University Medical School

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    目的 分析归纳胃肠道及系膜反应性结节状纤维假瘤(RNFPT)的临床、内镜和病理学特征及预后。 方法 将南京鼓楼医院2008年10月至2016年6月诊治的24例RNFPT患者纳入回顾性分析,对其临床表现、内镜特征、病理及免疫组化结果进行总结。 结果 24例患者中16例因消化道症状就诊,10例有手术或外伤史。21例为单发,3例为多发,直径0.5~4.0 cm。内镜下主要表现为广基黏膜下隆起,质地硬或韧,瘤体表面黏膜光滑,无黏膜桥形成。其中有17例行内镜超声检查,病变均呈低回声改变,部分区域回声欠均匀;肿瘤起源于固有肌层13例、黏膜下4例。光镜下肿瘤境界清楚,无包膜,主要表现为增生的梭形细胞无方向性杂乱排列伴广泛胶原变性的间质,大部分梭形细胞体积较小呈长梭形,核细长深染,无坏死,罕见核分裂象;其内散在慢性炎症细胞浸润,部分区可见淋巴滤泡形成,局灶区伴钙盐沉积。免疫组化染色显示,7例有SMA局灶阳性,4例有CD117极少量细胞散在阳性,Desmin、Dog-1、CD34、ALK-1、S-100均阴性,Ki-67增殖指数均低于1%。随访期内均无复发及转移。 结论 RNFPT临床表现各异,内镜以及病理形态需与梭形细胞肿瘤鉴别,其预后良好、不易复发。

    Abstract:

    Objective To study the clinical, endoscopic and pathological features of gastrointestinal and mesenteric reactive nodular fibrous pseudotumor (RNFPT). Methods A retrospective analysis was conducted on data of 24 RNFPT patients in Nanjing Drum Tower Hospital admitted from October 2008 to June 2016. The clinical, endoscopic, pathological and immunohistochemical characteristics were analyzed. Results Among the 24 patients, 16 complained about discomfort in the upper abdomen and 10 had a history of surgery or trauma. Twenty-one had isolated masses and 3 had multiple masses, with diameter of 0.5-4.0 cm. Endoscopically, the tumors were mainly hard submucosal masses with broad base, and smooth surface with no mucosal bridge. Seventeen patients underwent endoscopic ultrasonography, which showed low echoes in lesions and nonuniform echoes partly. Among them, 13 lesions derived from muscularis, 4 others from submucosa. Microscopically, the tumors had clear boundary with no envelope, and most areas showed disorderly arranged spindle cells and extensively collagenous degenerated mesenchyma. The spindle cells had shuttle fibroblast-like morphology and elongated nucleus with no visible necrosis or mitosis. Inflammatory cells scattered between the tumor cells, and lymphoid follicles and calcium deposition could be seen in local areas. Immunohistochemically, SMA was focally positive in 7 cases and only 4 cases expressed CD117 scattered. Desmin, Dog-1, CD34, ALK-1 and S-100 were all negative, and Ki-67 proliferation index was lower than 1%. Conclusion RNFPT has diverse clinical manifestations, with a good prognosis and unlikely recurrences, and should be distinguished from spindle cell tumors.

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王建军,王显香,吕瑛,等.胃肠道及系膜反应性结节状纤维性假瘤临床、内镜及病理特征分析[J].中华消化内镜杂志,2018,35(12):876-879.

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  • 收稿日期:2018-03-12
  • 最后修改日期:2018-10-20
  • 录用日期:2018-07-05
  • 在线发布日期: 2019-01-02
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