中华小儿外科杂志
中華小兒外科雜誌
중화소인외과잡지
CHINESE JOURNAL OF PEDIATRIC SURGERY
2013年
12期
882-886
,共5页
孟庆娅%张辉%胡晓丽%詹江华
孟慶婭%張輝%鬍曉麗%詹江華
맹경아%장휘%호효려%첨강화
肌纤维瘤病%免疫组织化学%诊断%治疗
肌纖維瘤病%免疫組織化學%診斷%治療
기섬유류병%면역조직화학%진단%치료
Myofibromatosis%Immunohistochemisty%Diagnosis%Treatment
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.
目的 探討兒童肺外炎性肌纖維母細胞瘤(inflammtory myofibroblastic tumor,IMT)的診斷及治療方法,進一步提高對于該疾病的認識.方法 迴顧性分析2008年1月至2012年12月間在我院外科收治的經病理檢查證實為肺外IMT患兒14例的臨床資料,總結其臨床錶現、診斷及治療方法,併結閤病理結果探討其疾病髮展過程.結果 14例患兒中無典型臨床癥狀,其臨床錶現依據髮病部位不同而有所區彆.髮病部位分彆位于腸繫膜、腹膜後、四肢、揹部、臀部、頭皮及附睪等,影像學檢查支持臨床診斷.14例均手術完整切除,術後2例跼部複髮,二次手術後結閤全身化療好轉.病理檢查可見密集的梭形細胞,伴炎細胞浸潤.免疫組織化學檢測SMA(+)13例,弱暘性1例;ALK(+)11例;desmin(+)10例;CD68(+)5例;CD34(+)2例;S-100(+)1例;1例Ki-67暘性＞15％.本組病人隨訪4箇月～4年,除1例失訪外,全部存活.結論 IMT是低度噁性軟組織腫瘤,臨床癥狀不典型,主要依靠病理診斷.兒童IMT中ALK錶達特異性高,手術切除是唯一可靠的治療手段,跼部複髮病人配閤全身化療可穫得較好療效.
목적 탐토인동폐외염성기섬유모세포류(inflammtory myofibroblastic tumor,IMT)적진단급치료방법,진일보제고대우해질병적인식.방법 회고성분석2008년1월지2012년12월간재아원외과수치적경병리검사증실위폐외IMT환인14례적림상자료,총결기림상표현、진단급치료방법,병결합병리결과탐토기질병발전과정.결과 14례환인중무전형림상증상,기림상표현의거발병부위불동이유소구별.발병부위분별위우장계막、복막후、사지、배부、둔부、두피급부고등,영상학검사지지림상진단.14례균수술완정절제,술후2례국부복발,이차수술후결합전신화료호전.병리검사가견밀집적사형세포,반염세포침윤.면역조직화학검측SMA(+)13례,약양성1례;ALK(+)11례;desmin(+)10례;CD68(+)5례;CD34(+)2례;S-100(+)1례;1례Ki-67양성＞15％.본조병인수방4개월～4년,제1례실방외,전부존활.결론 IMT시저도악성연조직종류,림상증상불전형,주요의고병리진단.인동IMT중ALK표체특이성고,수술절제시유일가고적치료수단,국부복발병인배합전신화료가획득교호료효.
Objective 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.
