摘要目的 探讨儿童肺外炎性肌纤维母细胞瘤(inflammtory myofibroblastic tumor,IMT)的诊断及治疗方法,进一步提高对于该疾病的认识.方法 回顾性分析2008年1月至2012年12月间在我院外科收治的经病理检查证实为肺外IMT患儿14例的临床资料,总结其临床表现、诊断及治疗方法,并结合病理结果探讨其疾病发展过程.结果 14例患儿中无典型临床症状,其临床表现依据发病部位不同而有所区别.发病部位分别位于肠系膜、腹膜后、四肢、背部、臀部、头皮及附睾等,影像学检查支持临床诊断.14例均手术完整切除,术后2例局部复发,二次手术后结合全身化疗好转.病理检查可见密集的梭形细胞,伴炎细胞浸润.免疫组织化学检测SMA(+)13例,弱阳性1例;ALK(+)11例;desmin(+)10例;CD68(+)5例;CD34(+)2例;S-100(+)1例;1例Ki-67阳性＞15％.本组病人随访4个月～4年,除1例失访外,全部存活.结论 IMT是低度恶性软组织肿瘤,临床症状不典型,主要依靠病理诊断.儿童IMT中ALK表达特异性高,手术切除是唯一可靠的治疗手段,局部复发病人配合全身化疗可获得较好疗效.

abstractsObjective To report the experience of diagnosis and treatment for extra-pulmonary inflammtory myofibroblastic tumor (IMT) in children.Methods From Jan.2008 to Dec.2012,fourteen patients(male 10,female 4) with extra-pulmonary IMT were operated on in the Department of Surgery,Tianjin Children's Hospital.The samples were confirmed by pathology.Clinical manifestations,diagnosis and treatment of IMT were analyzed.Results The sites of extra-pulmonary IMT included abdominal cavity,retroperitoneal cavity,extremities,head and neck,buttocks,scalp and epididymis.Two cases had local recurrence after operation.No distant metastases occurred.Histopathology revealed a spindle cell lesion,accompanied by inflammatory cell infiltration.Immunohistochemistry ALK was positive in 11/14 and SMA in 13/14,CD68 in 5/14,desmin in 10/14,CD34 in 2/14,S-100 in 1/14 and Ki-67 in 1/14.All patients were alive after surgery except that one patient lost follow-up.Conclusions IMT is a low-grade malignant tumor in Children.Clinical symptoms are not typical.The diagnosis mainly relies on pathology and immunohistochemistry results.ALK is expressed specifically in IMT samples.Surgery is the only effective treatment for children with IMT.For cases with local recurrent,surgery combined with chemotherapy would achieve good results.