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1、先天性纖維肉瘤的臨床病理分析及文獻復習黃海花1,吳秀淺2,張薇1,汪洪志1,鄭志超11汕頭大學醫(yī)學院第二附屬醫(yī)院病理科;2汕頭大學醫(yī)學院附屬腫瘤醫(yī)院介入科目的:探討先天性纖維肉瘤的臨床病理改變、分子遺傳學特點、診斷、鑒別診斷、治療及預后。方法:分析3例先天性纖維肉瘤的臨床資料、組織形態(tài)學特點及免疫組織化學表型,結(jié)合文獻復習。結(jié)果:三個病例的原發(fā)部位分別為耳廓、左前臂及腹膜后,發(fā)病年齡均在5歲以內(nèi),術(shù)前均未能明確診斷,其中一-例誤診為血管瘤,治療以手術(shù)切除為主,其中一例術(shù)前冇化療,隨訪至今,除一例局部復發(fā)行截肢術(shù)外,其余兩例未發(fā)現(xiàn)復發(fā)或轉(zhuǎn)移。腫瘤細胞均表達Vimentin
2、,分表達SMA或AAT。結(jié)論:先天性纖維肉瘤是發(fā)生于嬰幼兒的少見的軟組織腫瘤,具冇獨特的分子遺傳學改變,治療以局部擴大切除加化療為主,雖易復發(fā),但預后較好。關(guān)鍵詞先大性纖維肉瘤;臨床病理;診斷;治療;預后中圖分類號:R730.262Congenitalfibrosarcoma:aclinicalpathologicalstudywithliteraturereviewHaihuaHuang【作者簡介】黃?;ǎ?975.),女,博士,副主任醫(yī)師,主要從事腫瘤的浸潤和轉(zhuǎn)移研究。木課題受廣東省自然基金及汕,XiuqianWu頭市重點科技項目資助,項目編號分別為粵科基辦字[20
3、10]3號10151503102000029和汕府科[2009]70號?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訂efibrosarcomahasthesolefeaturesofmoleculargeneticsalteration.Therefore,completelylocalresectionorLocalexpandresectionwithchemotherapyisalwaysgivenprioritytothetreatment,andtheprognosisisgoodthoughiteasyrelapse.Keyword