CAJ | 학술논문

目的探讨间变性淋巴瘤激酶(ALK)蛋白在肺外炎性肌纤维母细胞瘤(IMT)中的表达情况,并分析其与临床复发之间的关系.方法回顾性分析2008年1月1日至2012年12月31日期间在我院外科诊治并经病理诊断的肺外IMT 14例,分别采用免疫组织化学技术Envision法染色和双色原位杂交技术,了解本组患儿标本中ALK表达情况,分析ALK阳性表达与局部复发之间的关系,确定ALK表达的临床意义.结果本组14例中,男10例,女4例;年龄2个月～13岁;肿瘤最大30 cm×25 cm×15 cm,来源于腹膜后;肿瘤最小0.8cm×0.8cm×0.5cm,来源于膝部.免疫组织化学结果:ALK(+)11例,同时行FISH检测,其中有2例发现有ALK基因重排,都是局部复发病例.再次手术切除复发病变,其免疫组织化学ALK仍为阳性表达.术后辅以化疗,随访2年未见复发.结论 IMT发病率较低,手术切除是治疗IMT的可靠方式;ALK蛋白表达可以证实IMT诊断,而ALK基因重排与肿瘤局部复发可能相关.
목적 탐토간변성림파류격매(ALK)단백재폐외염성기섬유모세포류(IMT)중적표체정황,병분석기여림상복발지간적관계.방법 회고성분석2008년1월1일지2012년12월31일기간재아원외과진치병경병리진단적폐외IMT 14례,분별채용면역조직화학기술Envision법염색화쌍색원위잡교기술,료해본조환인표본중ALK표체정황,분석ALK양성표체여국부복발지간적관계,학정ALK표체적림상의의.결과 본조14례중,남10례,녀4례;년령2개월～13세;종류최대30 cm×25 cm×15 cm,래원우복막후;종류최소0.8cm×0.8cm×0.5cm,래원우슬부.면역조직화학결과:ALK(+)11례,동시행FISH검측,기중유2례발현유ALK기인중배,도시국부복발병례.재차수술절제복발병변,기면역조직화학ALK잉위양성표체.술후보이화료,수방2년미견복발.결론 IMT발병솔교저,수술절제시치료IMT적가고방식;ALK단백표체가이증실IMT진단,이ALK기인중배여종류국부복발가능상관.
Objective To explore the anaplastic lymphoma kinase (ALK) expression in extrapulmonary inflammatory myofibroblastic tumor (IMT) and analyze its relationship with clinical local recurrence.Methods Retrospective clinical data reviews were performed for 14 cases of inflammatory myofibroblastic tumor at Tianjin Children's Hospital from January 1,2008 to December 31,2012.Irnmunohistochemical technology Envision staining and dual-color fluorescencein in situ hybridization (FISH) were employed to determine the ALK expression in whole specimens and analyze the relationship between clinical expression of ALK positive cells and local tumor recurrence.Results There were 10 males and 4 females with an age range of 2 months to 13 years.The largest diameter of retroperitoneal tumor was 30 cm× 25 cm× 15 cm and a minimal diameter of knew at 0.8 cm× 0.8 cm× 0.5 cm.The results of immunohistochemistry staining showed 11 cases of ALK (+).A further detection of FISH revealed 2 cases of ALK gene rearrangement as local recurrence.After resection of recurrent lesion,immunohistochemical staining ALK expression was still positive.Postoperative chemotherapy was administered.There was no recurrence during a follow-up of 2 years.Conclusions The incidence rate of IMT is low and its operation is reliable.And the expression of ALK protein may confirm its diagnosis and local tumor recurrence is probably related with ALK gene rearrangement.