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目的探讨儿童肠系膜及网膜炎性肌纤维母细胞肿瘤(IMT)的临床病理特征。方法收集5例肠系膜及网膜IMT儿童的临床资料,分析和总结其主要临床表现、辅助检查、病理学诊断依据及治疗方法。结果5例儿童肠系膜及网膜IMT临床均表现为腹腔内包块,其中2例腹痛,1例腹胀,1例呕吐,5例均有贫血及白细胞、血小板和血球蛋白增多。病理学检查:4例为黏液型IMT,1例为梭形细胞密集型IMT。免疫组织化学示梭形细胞呈波形蛋白、平滑肌肌动蛋白、结蛋白阳性表达,间变型淋巴瘤激酶和细胞角蛋白部分阳性表达,S-100蛋白、CD34均为阴性。5例肿瘤均完整切除,临床随访5个月～3a,无复发。结论儿童肠系膜及网膜IMT是一种较为罕见的间叶性肿瘤,诊断必须依靠病理学检查和免疫组织化学检测。肿瘤大多为良性,完整的手术切除可有效预防局部复发。
Objective To investigate the clinicopathological features of mesenteric and omental inflammatory myofibroblastic tumors (IMT) in children. Methods The clinical data of 5 cases of IMT in the mesentery and omentum were collected and analyzed. The main clinical manifestations, auxiliary examinations, the basis of pathological diagnosis and treatment were analyzed and summarized. Results All the 5 cases of mesenteric and omental IMT showed intraperitoneal mass, including 2 cases of abdominal pain, 1 case of abdominal distension and 1 case of vomiting. All 5 cases had anemia and increased leukocyte, platelet and hemoglobin. Pathological examination: 4 cases of mucinous IMT, 1 case of spindle-cell-dense IMT. Immunofluorescence showed that the spindle cells were positive for vimentin, smooth muscle actin, desmin, anaplastic lymphoma kinase and cytokeratin, and S-100 protein and CD34 were all negative. 5 cases of tumor were completely resected, clinical follow-up of 5 months to 3a, no recurrence. Conclusion Mesenteric and omental IMT in children are a rare type of mesenchymal tumor. The diagnosis must rely on pathological examination and immunohistochemistry. Tumors are mostly benign, complete surgical resection can effectively prevent local recurrence.