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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
