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1、Malignantperipheralnervesheathtumors(惡性外周神經(jīng)鞘瘤)-Malignantperipheralnervesheathtumors(MPNST)areanuncommonbutdevastatingtumorofperipheralnerve,representingonlyabout10percentoftumorsencounteredbyaperipheralnervesurgeon[43].TheincidenceofMPNSTsinthegeneralpopula
2、tionis0.001percent.MPNSTs,whichareclassifiedasmalignantsofttissuesarcomas,canarisefrompre-existingplexiform叢狀neurofibromasorperineuriomas(神經(jīng)束膜瘤)ornormalnerves.Theydonotariseformschwannomas(神經(jīng)鞘瘤).MPNSTsalsooccurassecondaryneoplasms10to20yearsafterradiationth
3、erapy.Accountingforupto10percentof???。。。。。Photo5MPNSTs[4],From22to50percentofMPNSTsoccurinpatientswithneurofibromatosis多發(fā)性神經(jīng)纖維瘤type1(NF1),therestbeingsporadic[2.43,44].TheriskofdevelopingaMPNSTinapatientwithNF1isbetween8and13percent[45],withmost,ifnotall,ar
4、isingfrompre-existingplexiformneurofitbromas(叢狀神經(jīng)纖維瘤（plexiformneurofibroma）：此瘤主要由神經(jīng)軸柱組成，多見于面部、頸后、上瞼等處，腫瘤可沿神經(jīng)及其分支向各方面發(fā)展，累及范圍廣，并可沿脊神經(jīng)進(jìn)入脊髓)[46].InpatientswithNF1,thepresenceofaninternalplexiformneurofitbromaisassociatedwitha20-foldincreasedriskofdevelopingaMPN
5、STcomparedwiththeriskinthoselackinganinternalplexiformneurofitbroma[46].MPNSTstendtopresentatanearlierageinpatientswithNF1(thirdorfourthdecadeoflife,versusseventhdecadeinpatientswhodonothaveNF1)[45].·MPNSTsaremostcommonlyfoundontheextremitiesandtrunk,andles
6、softenontheheadandneck[47].Theclinicalpicture臨床現(xiàn)象ischaracterizedbyrapidchange,whetherinpain,sizeoftumormass,orprogressionofneurologicdeficit(神經(jīng)缺陷,神經(jīng)痛),especiallywhenoccurringinapre-existingperipheral周圍nervetumor[2].Inparticular,frequentsitesofinvolvement[43
7、],butanerveoforiginisoftennotevident[2].Thediagnosisofmalignant惡性versusbenigh良性tumorrequiresbiopsy活檢,whichshouldbeopenandinvolvemultiplesectionsofthetumor[6.43].Conventionalradiographicimagingmayhelpbutcannotreliablydifferentiatebetweenmalignantandbenign,la
8、rgetumorsize(>5cm),heterogeneity,ill-definedmargins不清晰的邊界,invasionoffatplanes脂肪層,andsurroundingedema（[病理]水腫（等于oedema）；瘤腺體）allsuggestMPNST[7](image2).AtleasesomedatasuggestthatPETimagingcanbehelpfulindi
