55例儿童横纹肌肉瘤的临床和病理特征.pdfVIP

上海交通大学学报 (医学版) ·1222 · JournalofShanghaiJiaotongUniversity(MedicalScience) Vol.26No.11Nov.2006 [文章编号] 0258一5898(2006)11一1222-03 ·专题报道 · 55例儿童横纹肌肉瘤的临床和病理特征 王捍平，严文波，吴 燕，沈涤华，奚政君，施诚仁，蔡 威 (上海交大医学院新华医院小儿外科，上海 200092) 【摘 要】 目的分析儿童横纹肌肉瘤(RMS)的临床和病理特征，提高早期诊治水平。方挂对55例RMS患儿的临床资料进行 分析。祛果 55例患儿中，学龄前儿童占76.4%。肿瘤部位:原发于头面部5例，四肢和躯干部12例，生殖泌尿系统24例，胸腹 腔14例。病理类型:胚胎型49例，腺泡型5例，多形型1例。头面部、四肢和躯干RMS表现为肿块，就诊时很少有伴发症状。 胸、腹、盆腔部位肿瘤早期无症状，肿瘤较大时产生占位和侵蚀引起的非特异性临床症状。就诊时恶液质少见。MRI或CT检查 的27例，第一诊断准确率为81%(22/27)o倍格 儿童RMS多发病于学龄前;绝大多数为预后相对较好的胚胎型;胸、腹、盆腔 部位RMS多表现非特异性症状;MRI或CT可初步诊断;确诊依靠肿瘤活检和免疫组化检测。 【关键词】横纹肌肉瘤;临床特征;病理学特征;儿童 【中图分类号】R73 [文献标识码】A ClinicalandPathologicalCharacteristicsof55ChildrenwithRhabdomyosarcoma WANGHan-ping，YANWen-bo，WUYan，SHENDi-hua，XIZheng一un，SHICheng-ren，CAI-wei (DepartmentofPediatricSurgery，XinhuaHospital，SchoolofMedicine，ShanghaiJiaotongUniversity，Shanghai 200092，China) Abstract:Objective Toimproveearlydiagnosisandtreatmentofrhabdomyosarcoma(RMS)byanalyzingclinical andpathologicalcharacteristics. Methods Theclinicaldatawereretrospectivelyanalyzedin55childrenwith RMS. w ere preschoolchildren w ere Results 76.4% patients Theprimarytumorsoffivecases incephalofacial site，12inlimbsandtrunk，24ingenmto-unnary system，and14insplanchnocoele，respectively.Forty-ninecases w ere Masswasthem ain pathologicallyclassifiedintoembryonicRMS，fiveacinarRMSandonepolymorphousRMS. presentationwithoutotherconcomitantsymptomsincephalofacialsite，limbsandtrunk.However,nonspecificclini- calfindingscouldbeobservedinthosewitherosion andoccupationofthem crescenttumorslocatedinsplanchnocoele orpelvis.DyscrasiawasrareinthecaseswithRMS.RMSwasinitiallydiagnosedin81%ocasesonwhom eitherMRI orCTscanwereperformed. Conclusion ChildhoodRMSisusuallyoccurredin