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1、HowtoTreattheEwing’s【关键词】Ep;#8226;,Adult,Prognosis,#8226;,Survival LEARNINGOBJECTIVES Afterpletingthiscourse,thereaderitiveneuroectodermaltumor,andAskin’stumorpriseasinglefamilyoftumors,theEilyoftumors,osomaltranslocation.Ealignancyofchildhood,butedian
2、ageof15yearsatdiagnosis,itshouldequallyberegardedasamalignancyofadolescenceandyoungadulthood.Thereismuchcontroversyregardingtheroleofageatdiagnosis,estudiesshoanagenonpediatricpatientsinesentinthisgroupoftumors. INTRODUCTION In1921,JamesEalignantbonetu
3、morposedofsmallroundcellsandnamedtheconditiondiffuseendothelioma[1].ItisnoilycalledtheEilyoftumors(ESFT),itiveneuroectodermaltumor(P),extraosseousEor(ESofthechestalignancyofchildhoodandadolescence.Itisthesecondmostmonbonecancerofchildhood[2,3],accounting
4、for42%ofcases[3];inthosepatientsedianageatdiagnosisofESaccordingtotheNationalCancerInstitute’sSurveillanceEpidemiologyandEndResults(SEER)datafortheperiod1973to1987fortheperiod1968to1995foundthat56%ofthepatientsatelyonehalfofallpatientsdiagnosedorportantt
5、orememberthatitisequallyadiseaseofyoungadultsasmuchasitisadiseaseofchildren. Thereisa"thatolder/adultpatientsfareworsethantheyoungerpatients,andthishasledtouncertaintyastotheappropri678910111213atemanagementforountstoasignificantproportionoftheEportanti
6、mplicationofthisisthepotentialriskthatyoungadultsmaynotbereceivingoptimaltreatment,eitherbecausetheydoinfacthaveaoreaggressivetreatmentorbecausetheyarenotbeingtreatedasaggressivelyasyoungerpatientsandasaresulthaveapooreroute. Ifolderpatientsdohaveapoore
7、route,isitadifferenceintheunderlyingbiology?Ordothesepatientshaveanexcessofotheradversefactors?Orarethesepatientsnotreceivingoptimaltreatmentbecauseofeitherinadequatedosingordifferingpharmacokiicsparedthis,makessuggestionsastoentofadultpatientsshoulddiff
8、erfromthatofyoungerpatientsors. BIOLOGYOFEILYOFTUMORS ESFTtumorsaretumorsofneuralcrestderivationthatdifferentiatealonganeuroendocrinelineageandaredescribedas"smallroundcelltumors."Althoughtherearelightmicroscopyvariation
