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1、先天性纤维肉瘤的临床病理分析及文献复习黄海花1，吴秀浅2,张薇1,汪洪志1,郑志超11汕头大学医学院第二附属医院病理科；2汕头大学医学院附属肿瘤医院介入科目的：探讨先天性纤维肉瘤的临床病理改变、分子遗传学特点、诊断、鉴别诊断、治疗及预后。方法：分析3例先天性纤维肉瘤的临床资料、组织形态学特点及免疫组织化学表型，结合文献复习。结果：三个病例的原发部位分别为耳廓、左前臂及腹膜后，发病年龄均在5岁以内,术前均未能明确诊断，其中一-例误诊为血管瘤，治疗以手术切除为主，其中一例术前冇化疗,随访至今，除一例局部复发行截肢术外，其余两例未发现复发或转移。肿瘤细胞均表达Vimentin
2、,分表达SMA或AAT。结论：先天性纤维肉瘤是发生于婴幼儿的少见的软组织肿瘤，具冇独特的分子遗传学改变，治疗以局部扩大切除加化疗为主，虽易复发，但预后较好。关键词先大性纤维肉瘤;临床病理；诊断；治疗；预后中图分类号：R730.262Congenitalfibrosarcoma:aclinicalpathologicalstudywithliteraturereviewHaihuaHuang【作者简介】黄海花（1975.）,女,博士，副主任医师，主要从事肿瘤的浸润和转移研究。木课题受广东省自然基金及汕,XiuqianWu头市重点科技项目资助，项目编号分别为粤科基办字[20
3、10]3号10151503102000029和汕府科[2009]70号・2,WeiZhang1,HongzhiWang1,ZhichaoZheng11DepartmentofPathology,SecondAffiliatedHospitalofMedicalCollegeofShantouUniversity,Shantou515041,GuangdongChina;2DepartmentofInterventionalTherapy,TumorHospitalofMedicalCollegeofShantouUniversity,Shantou515041，Guan
4、gdongChinaAbstractObjectives:Tostudytheclinicopathologicalalterations,moleculargeneticsfeatures,treatment,differentialdiagnosisandprognosisofcongenitalfibrosarcoma.Methods:Toanalysistheclinicalmaterials,morphologicalfeatures,immunochemistryphenotypcfrom3casescongeniteilfibrosarcoma,andre
5、viewtheliteratureonthissubject.ResuIts:Theprimarysiteof3casesisauricle,leftforearmandretroperitonealrespectively.Theageofthesepatientswaslessthan5yearsold.Preoperativediagnosisisnotaccurate,oneinitiallymisdiagnosedwithhemangioma.Treatmentismainlybythesurgery,onecasereceivedchemotherapybe
6、foreoperation.Uptodata,thosethreecaseswereanalyzedandfollowedup.Exceptforonecasereceivedpartialcomplexeimputation,theothersdidnotfoundrecurrenceormetastasis.TheexpressionofVimentinwasfoundinallthealltumorcells,theexpressionofSMAorAATwasfoundinsomecells.Conclusion:Asararesofttissuemaligna
7、ncyoccursinaxialandextremitylocations，congenitalinfant订efibrosarcomahasthesolefeaturesofmoleculargeneticsalteration.Therefore,completelylocalresectionorLocalexpandresectionwithchemotherapyisalwaysgivenprioritytothetreatment,andtheprognosisisgoodthoughiteasyrelapse.Keyword
