how to treat the ewing’s family of saras in adult patients

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1、HowtoTreattheEwing’sFamilyofSarasinAdultPatients　　Describehoitiveneuroectodermaltumor,andAskin’stumorpriseasinglefamilyoftumors,theEilyoftumors,osomaltranslocation.Ealignancyofchildhood,butedianageof15yearsatdiagnosis,itshouldequallyberegardedasamalignancyofadolescenceandyoungadulthood.Thereismuchc

2、ontroversyregardingtheroleofageatdiagnosis,estudiesshoanagenonpediatricpatientsinesentinthisgroupoftumors.　　INTRODUCTION　　In1921,JamesEalignantbonetumorposedofsmallroundcellsandnamedtheconditiondiffuseendothelioma[1].ItisnoilycalledtheEilyoftumors(ESFT),itiveneuroectodermaltumor(P),extraosseousEor(

3、ESofthechestalignancyofchildhoodandadolescence.Itisthesecondmostmonbonecancerofchildhood[2,3],accountingfor42%ofcases[3];inthosepatientsedianageatdiagnosisofESaccordingtotheNationalCancerInstitute’sSurveillanceEpidemiologyandEndResults(SEER)datafortheperiod1973to1987fortheperiod1968to1995foundthat5

4、6%ofthepatientsatelyonehalfofallpatientsdiagnosedorportanttorememberthatitisequallyadiseaseofyoungadultsasmuchasitisadiseaseofchildren.　　Thereisa"thatolder/adultpatientsfareanagementforountstoasignificantproportionoftheEportantimplicationofthisisthepotentialriskthatyoungadultsmaynotbereceivingoptim

5、altreatment,eitherbecausetheydoinfacthaveaoreaggressivetreatmentorbecausetheyarenotbeingtreatedasaggressivelyasyoungerpatientsandasaresulthaveapooreroute.　　Ifolderpatientsdohaveapooreroute,isitadifferenceintheunderlyingbiology?Ordothesepatientshaveanexcessofotheradversefactors?Orarethesepatientsnot

6、receivingoptimaltreatmentbecauseofeitherinadequatedosingordifferingpharmacokiicsparedthis,makessuggestionsastoentofadultpatientsshoulddifferfromthatofyoungerpatientsors.　　BIOLOGYOFEILYOFTUMORS　　ESFTtumorsaretumorsofneuralcrestderivationthatdifferentiatealonganeuroendocrinelineageandaredescribedas"s

7、mallroundcelltumors."AlthoughtherearelightmicroscopyvariationsbetorsarecharacterizedbyabalancedchromosomaltranslocationbetembersoftheETSfamilyoftranscriptionfactorshasledtotheunderstandingthattheESFTreprese