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1、萬方數(shù)據(jù)·776·中國腫瘤臨床炎性肌纖維母細胞瘤2007年第34卷第13期摘要炎性肌纖維母細胞瘤(IMT)是近年被認識和正式命名的獨立的中間型(低度惡性)腫瘤，涵蓋了以往從炎性反應性病變到腫瘤的一系列診斷。發(fā)生于肺和全身各處，表現(xiàn)局部腫塊，可伴有發(fā)熱、體重減輕、盜汗及淋巴結(jié)腫大等全身癥狀。病變以單發(fā)或多灶發(fā)生，局部浸潤性生長，侵犯血管，局部復發(fā)，少數(shù)病程進展快而致死。較大腫塊局部浸潤性生長臨床和影像學頗似惡性腫瘤。組織學顯示炎癥背景下不同數(shù)量纖維母細胞、肌纖維母細胞、淋巴細胞、漿細胞、嗜酸性粒細胞和組織細胞，間質(zhì)為粘液性、纖維血管
2、性或膠原性。病變可類似炎癥性反應性增生，也可出現(xiàn)壞死、細胞異型增生易與梭形細胞肉瘤／癌混淆。真正病因尚不清楚，已證實腫瘤內(nèi)肌纖維母細胞有間變性淋巴瘤激酶(ALK)基因重排和表達，并有ALK基因與Rb-2蛋白基因的融合，證實腫瘤性增生本質(zhì)。本文對該病的認識過程、遺傳學研究、病理學、影像學及臨床行為做一綜述。關鍵詞炎性肌纖維母細胞瘤中間型腫瘤診斷InflammatoryMyofibroblasticTumorCaoHaiguangReviewedbyLiuSuxiangENTDepartmentoftheTianjinFirstCen
3、tralHospital．TianjinInflammatorymyofibroblastictumor(IMT)hasbeenrecognizedandformallydescribedasaninter—mediatetypeoftumorwithlowpotentialmalignancy，comprisingarangeofdiagnosesfrompathologicalchangesinvolvedininflammatoryreactionstotumors．Itisfoundinallpartsofthehuman
4、body。usual-lypresentingwithaclinicalmanifestationofalocallumpandgeneralsymptomssuchasfever，weightloss，nightsweatsandlymphadenectasis，etc．Ithasunifocalormuhifoealsymptomswithacapacityforregionalinfiltrativegrowth，vascularinvasion，localrecurrenceandevenrapiddevelopment，
5、causingac—celerateddeathinafewcases．Thetumorscanbeofconsiderablesizeandarecapableofinfiltrativegrowth，mimickingamalignanttumorclinicallyandradiologically．Myofibroblast，lymphocyte，plasmo—cyte，eosinophilgranulocyte，histiocyte，mucousinterstitium，andvascularorcollagenousf
6、iberinvary—ingamountscanbefoundmicroscopically．Thesefeaturesmimicinflammatoryreactivehyperplasiaandalso，butlessSO，necrosis，cellulardysplasiaandfascicularsarcoma．Theexactetiologyofthislesionisunknown．Thereisexpressionofanaplasticlymphomakinase(ALK)inIMTresultingfromafu
7、sionoftheALKgenetotheRan—bindingprotein2(RANBP2)gene．Thediagnosticprocess，genetics，patholo—gy，radiology，andclinicalbehaviorofIMTisdescribedherein．KeywordsInflammatorymyofibroblastomaIntermediatetypetumorDiagnosis1概述炎性肌纖維母細胞瘤(Inflammatorymyofibroblastictumor．IMT)是一種少見而
8、獨特的間葉性腫瘤．表現(xiàn)低度惡性或交界性腫瘤特點，近年通訊作者：曹海光hg—c@163．netWHO提出此命名。已逐漸得到廣泛認同[1，2】。IMT多發(fā)生于肺，也見于頭頸、軀干、內(nèi)臟及四肢軟組織。由于臨床及影像學表現(xiàn)侵襲性占位性病變，組織形態(tài)變化多樣
