儿童肝脏未分化胚胎性肉瘤诊治分析-论文.pdf

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1、·412·J0URNALOFBASICANDCLINICALONCOLOGYVo1．26No．5Oct．2013儿童肝脏未分化胚胎性肉瘤诊治分析侯维纳，邹湘，郭佳，马莹莹，王璐，盛光耀，孙素珂(1．郑州大学第一附属医院儿科，河南郑州450052；2．河南省高等学校临床医学重点开放实验室，河南郑州450052)[摘要]目的探讨儿童肝脏未分化胚胎性肉瘤(UESL)的临床表现、诊断、治疗及预后特点。方法回顾2例儿童UESL并复习相关文献，从临床表现、影像学、实验室检查、治疗及预后方面对UESL进行综合分析。结果UESL主要见于5—10岁儿

2、童。UESL的临床表现多样且无特异性，常见的临床表现为腹部肿块和腹痛。实验室检查也无特异性，肝功能、AFP多正常。影像学检查示肝脏巨大占位，瘤体多位于肝右叶，B超与CT表现的不一致性是本病的重要特点。确诊有赖于组织病理学和免疫组化检查。UESL预后较差，目前还无公认的UESL的标准治疗方案，手术、术前或术后的化疗是现行治疗方案。结论UESL是一种高度恶性肿瘤，做组织病理学检查之前，诊断困难，误诊率高，预后较差。肝脏巨大占位，AFP正常，B超与CT表现不一致，要考虑UESL的诊断。手术完整切除，同时辅以多药化疗联合治疗可明显提高患儿生

3、存率。UESL需早诊断、早治疗，因为UESL是一种潜在可治疗肿瘤。[关键词]肝脏；未分化胚胎性肉瘤；儿童DOI：10．3969／j．issn．1673-5412．2013．05．013[中图分类号]R735．7[文献标识码]A[文章编号]1673—5412(2013)05—0412—04DiagnosisandTreatmentofUndiferentiatedEmbryonalSrcomaoftheLiverinChildrenHouWeina，ZouXiang，GnoJia，MaYingying，WangLu。ShengGuan

4、gyao，SunSuke，(1．DepartmentofPediatrics，theFirstAfiliatedHospitalofZhengzhouUniversity，Zhengzhou450052，China；2．KeyenLaboratoryofHenanClinicalMedicine，Zhengzhou450052，China)[Abstract]0bjectiveToexploretheclinicalmanifestations，diagnosis，treatmentandprognosisofundifferent

5、i—atedembryonalsarcomaoftheliver(UESL)inchildren．MethodsTwocasesofUESLinchildrenwereretrospec—tivelyanalyzed，andtheliteraturewasrenewed，thecomprehensiveanalysisofUESLfromtheclinicalmanifesta—tions，imagingfindings，laboratorydata，treatmentandprognosiswerereceived．Results

6、UESLwasprimarilyseenbetweentheagesof5and10yearsold．TheclinicalfeaturesofUESLwerevariedandoftennonspecific．Commonpresentingfeaturesincludedlargeabdominalmassandabdominalpain．Laboratorystudieswereusuallynonspecificaswel1．TheyusuallyshowednoabnormalitiesinliverfunctionorA

7、FPleve1．Imagingexaminationusuallyshowedthelesionoccupyingalargespaceintheliver，therightlobeoftheliverwasmorecommonlyinvolved．DiscrepancyofimagingbetweenultrasoundandCTwasoneoftheimportantcharacteristicsofUESL．Diagnosisdependedonhis—topathologicalandimmunohistochemicale

8、xaminationofthemass．TheprognosisofUESLwaspoor，nowtherewasnostandardtreatmentmentionedintheliterature，surgerycombinedw