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1、Malignantperipheralnervesheathtumors(恶性外周神经鞘瘤)-Malignantperipheralnervesheathtumors(MPNST)areanuncommonbutdevastatingtumorofperipheralnerve,representingonlyabout10percentoftumorsencounteredbyaperipheralnervesurgeon[43].TheincidenceofMPNSTsinthegeneralpopula
2、tionis0.001percent.MPNSTs,whichareclassifiedasmalignantsofttissuesarcomas,canarisefrompre-existingplexiform丛状neurofibromasorperineuriomas(神经束膜瘤)ornormalnerves.Theydonotariseformschwannomas(神经鞘瘤).MPNSTsalsooccurassecondaryneoplasms10to20yearsafterradiationth
3、erapy.Accountingforupto10percentof???。。。。。Photo5MPNSTs[4],From22to50percentofMPNSTsoccurinpatientswithneurofibromatosis多发性神经纤维瘤type1(NF1),therestbeingsporadic[2.43,44].TheriskofdevelopingaMPNSTinapatientwithNF1isbetween8and13percent[45],withmost,ifnotall,ar
4、isingfrompre-existingplexiformneurofitbromas(丛状神经纤维瘤（plexiformneurofibroma）：此瘤主要由神经轴柱组成，多见于面部、颈后、上睑等处，肿瘤可沿神经及其分支向各方面发展，累及范围广，并可沿脊神经进入脊髓)[46].InpatientswithNF1,thepresenceofaninternalplexiformneurofitbromaisassociatedwitha20-foldincreasedriskofdevelopingaMPN
5、STcomparedwiththeriskinthoselackinganinternalplexiformneurofitbroma[46].MPNSTstendtopresentatanearlierageinpatientswithNF1(thirdorfourthdecadeoflife,versusseventhdecadeinpatientswhodonothaveNF1)[45].·MPNSTsaremostcommonlyfoundontheextremitiesandtrunk,andles
6、softenontheheadandneck[47].Theclinicalpicture临床现象ischaracterizedbyrapidchange,whetherinpain,sizeoftumormass,orprogressionofneurologicdeficit(神经缺陷,神经痛),especiallywhenoccurringinapre-existingperipheral周围nervetumor[2].Inparticular,frequentsitesofinvolvement[43
7、],butanerveoforiginisoftennotevident[2].Thediagnosisofmalignant恶性versusbenigh良性tumorrequiresbiopsy活检,whichshouldbeopenandinvolvemultiplesectionsofthetumor[6.43].Conventionalradiographicimagingmayhelpbutcannotreliablydifferentiatebetweenmalignantandbenign,la
8、rgetumorsize(>5cm),heterogeneity,ill-definedmargins不清晰的边界,invasionoffatplanes脂肪层,andsurroundingedema（[病理]水肿（等于oedema）；瘤腺体）allsuggestMPNST[7](image2).AtleasesomedatasuggestthatPETimagingcanbehelpfulindi
