Objective To explore the clinicopathological features, immune phenotype, the differential diagnosis of gastrointestinal schwannomas (GSs). Methods The clinical data, pathological morphology and immune phenotype of 4 cases of GSs were observed, discussed and analyzed by combining with literature. Results The 4 patients, included 3 women and 1 man, mean age (61.3±9.9) years, with no specific clinical signs and symptoms. Imaging tumor showed clear bounded slightly low density homogeneous entity foci. The tumor had no capsule, the boundary was clear, the cross section was solid, glossy via gross observation. Under the micro-scope, lymphocyte sets, spindle tumor cells formed by rich lymphocytes proliferation in periphery of tumor tissue arranged in cross-ing fascicles, the nuclei in local area were not significant palisade, could have a significant focal nuclear atypia, but mitotic rare. Focal region of the tumor margin showed the tumor cells separating, wrapping the stomach muscle phenomenon. Immunohisto-chemistry showed that 4 cases of tumor cells S-100, Vimentin showed diffuse strong positive, 2 cases of focal piece GFAP positive, Ki-67 positive index was less than 3%, Desmin, CD34, CD117, DOG-1 were negative. Postoperative follow-up showed no recur-rence or metastasis. Conclusion GSs is a rare gastrointestinal mesenchymal benign tumor, grasping the relative characteristic pathologic morphology combined with S-100, CD117, CD34, DOG-1 and other markers contribute to the diagnosis and differential diagnosis.%目的：探讨胃肠道神经鞘瘤（GSs）临床病理特征、免疫表型、鉴别诊断。方法对4例GSs患者临床资料、病理形态、免疫表型进行观察，结合文献进行讨论分析。结果4例患者，平均年龄（61.3±9.9）岁，女3例，男1例。患者无特殊临床症状和体征。影像学瘤体表现为界清的稍低密度均质实体灶。大体观察肿瘤无包膜，界清，切面呈实性，有光泽感。镜下瘤组织外围均有丰富的淋巴细胞增生形成的淋巴细胞套，梭形瘤细胞呈交叉束状排列，局部区域细胞核呈不显著的栅栏状，局灶可有显著的核异型性，但核分裂罕见。肿瘤边缘局灶区域可见瘤细胞分隔、包裹胃壁平滑肌现象。免疫组化显示4例瘤细胞S-100、Vimentin呈弥漫强阳性，2例GFAP片灶性阳性，Ki-67阳性指数＜3%，Desmin、CD34、CD117、DOG-1均为阴性。术后随访均未见复发或转移。结论 GSs 是胃肠道罕见的间叶源性良性肿瘤，掌握其相对特征性病理学形态结合 S-100、CD117、CD34、DOG-1等标记物检测有助于诊断和鉴别诊断。
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