作者单位 复旦大学附属眼耳鼻喉科医院病理科 上海,3; 复旦大学附属肿瘤医院病理科 上海,3通讯作者 王纾宜,2y@·论著·文章编号:167325501(2008)0520350206儿童头面部胚胎性横纹肌肉瘤的临床病理特点及预后分析林 岚1 王纾宜1 王 坚2 摘要 目的 探讨儿童头面部胚胎性横纹肌肉瘤的临床病理特征及其与预后的关系。
方法 按2002年国际病理学会的诊断标准收集复旦大学附属眼耳鼻喉科医院病理诊断确诊的头面部胚胎性横纹肌肉瘤病例,并将葡萄状型归入胚胎性的亚型。
收集病理及免疫组化切片及临床资料,并请复旦大学附属肿瘤医院病理科软组织专家重新阅读病理片,对发生于儿童头面部的胚胎性横纹肌肉瘤进行临床病理学分析。
结果 2004～2007年共有18例患儿纳入分析,其中男12例,女6例;发病年龄5个月至18岁,<10岁者12例。
临床表现为口、鼻或耳道肿物及鼻塞、声嘶、面瘫等肿瘤占位引起的相应症状和体征。
依据美国横纹肌肉瘤研究组的分期标准,临床Ⅰ～Ⅱ期7例,Ⅲ～Ⅳ期11例。
CT或MR I显示相应部位膨胀性生长的软组织占位,多伴有骨吸收破坏。
术前有5例临床诊断为良性病变。
组织病理学上,以出现不同分化阶段的横纹肌母细胞为特征,其中葡萄状亚型4例。
行肿瘤完整或大部切除5例,部分切除2例,11例仅行手术探查或鼻内镜活检。
随访到10例患儿,其中4例患儿病死,均为临床Ⅲ期,存活的6例均采用了手术结合化疗或放疗的综合治疗;余8例失访。
结论 儿童头面部胚胎性横纹肌肉瘤为高度恶性的软组织肿瘤,生长迅速,临床较易漏诊或误诊,早期正确诊断和综合治疗是提高生存率的关键。
关键词 胚胎性横纹肌肉瘤; 病理学; 免疫组织化学; 儿童C lin ico p a th o lo g ica l c h a ra c te ris tic s an d p ro g n o s is o f e m b ry o n a l rh a b d om yo s a rcom a o f th e h ea d in c h ild renL I N L an1,WAN G Shu2yi1,WANG J ian2(1D epa rt m ent of Pa thol ogy,EEN T Hospita l of Fudan U niv ersity,Sha ngha i200031, China;2D epa rt ment o f Pa thology,Tumor Hospit a l of Fudan U niv ersity,Sha ngha i200032,China)Co r res p ond ing A u th o r:WANG Shu2yi,E2m ail:wangshuyi@eent.sh m A b s tra c t O b jec ti ve E mbry onal rhabdo m y osarco m a(ERMS)is probably the most co mmon sarco ma a risi ng in the head and neck in children.The c linical behavi or of this pa rticular rhabdo m y osarco m a is kno wn to be highly aggressive,and its event ual outco m e is dis m al.Our pu r pose wa s t o study the clinicopa thol ogical characteristic s and t o discuss the co rrela ti on of c linicopathol ogical feature s of ERMS and progn osis.M e tho ds We conducted a re tros pective study of children diagn osed for embry onal rhabd omyos a rco ma of the head and neck a t EE NT Hos p ital of Fudan University bet ween the yea rs2004and2007in accordance wit h the diagn ostic crite ria of Internati onal Academy of Pathol og y(I A P2002)and botryoid rhabdo m y osarco m a(BERM S) was included in ERMS.The tu mours were st aged according t o the I ntergrou p R habdo m y osarco m a Study.The cli n i ca l,pathol ogical and i m mun ohist ochem ica l fea tures of18ca s e s of em bry onal rhabdomy o s a rcoma we re eva l uated.Re s u lts There were12b oys and6 girls,aged fr om5months t o18yea rs,among them,12ca ses we re y ounge r t han10years.C linica lly,most pati ents presented with a ma ss in t he mouth,nose or ea r regi ons and sy mp t o m s were re l a ted t o tu mor inva si on,such as na s a l obstruc tion,trachy phonia and fac ial para lysis.Seven case s belong ed to stageⅠ-Ⅱ,while11case s we re in stageⅢ-Ⅳ.CT scan or MR I usua lly sho wed a s oft tissue tu mor w ith ex pansi ve gr owth,assoc iated wit h bone e rosi on or de struction.Before surgery,5cases we re m is diagnosed as benign l e si ons.They were poly p s,inflamm ati on m ass,nas opharyngea l angi ofibr oma,cholest ea t oma and t y mpanic paragangli oma re s pecti ve ly.Hist ol ogica lly,the tumo rs consisted of a variable ad m ixture of undiffe rentia t ed and diffe rentiated ce lls (rhabdo m y obla sts).Among them,4cases belonged t o t he botry oid variant which ty p i ca lly had a cambiu m layer.The tu mor was t otally or subtotally removed in5case s,partia lly removed in2cases,and bi op sied only in11cases.Foll o w2up i nfor ma ti on of10 fⅢ,6y120001220002E ma il:w angshu i e pa tien ts sho w ed4ca ses d ied o d isease s belonged to stage an d ca ses a lived w ithou t d isea se ad op t ed mu lti moda l th erap w ithcombina tion of s urgery,che mothe rapy and radi a tion.C on c lus ions E mbry onal rhabdomy osa rcoma of t he head in children is a highly ma lignant tumor which gro ws quickly.In ea rly stage,it m ay be m isdi agnosed.Early and p rec ise diagnosis and co m bined manag em en t is crucia l t o i m p rove t he survival rate.Ke y w o rds Embryonal rhabdomy osa rcoma; Pa thol ogy; I mmun ohist oche m istry; Children 横纹肌肉瘤是儿童和青少年软组织肉瘤最常见的类型,占所有软组织肿瘤的60%[1],其发病率在儿童颅外肿瘤中位于神经母细胞瘤及W il m′s瘤之后,居第3位。
