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外科-骨癌的治疗ManagementofbonetumoursINTRODUCTIONBenignbonetumoursoutnumbertheirmalignanttumoursby3to4-fold.ThedistributionoftumoursvarieswithageMostbenignbonetumours,osteosarcomas,andEwing'ssarcomasoccurinthesecondandthirddecade.Giantcelltumours,chondrosarcomas,fibrosarcom...

外科-骨癌的治疗
ManagementofbonetumoursINTRODUCTIONBenignbonetumoursoutnumbertheirmalignanttumoursby3to4-fold.ThedistributionoftumoursvarieswithageMostbenignbonetumours,osteosarcomas,andEwing'ssarcomasoccurinthesecondandthirddecade.Giantcelltumours,chondrosarcomas,fibrosarcomas,myelomas,lymphomas,andmetastaticdiseaseallhaveapredilectionforolderagegroupsINTRODUCTIONWiththeexceptionofgiantcelltumours,MostbenignandmalignanttumoursofboneareslightlymorecommoninmenAnincreasedincidenceofsarcomasisseeninbonepreviouslyirradiatedNoevidencethatasingleuncomplicatedinjurycausessarcomasCLASSIFICATIONThetypesoftumoursoccurringwithinitscomponentsOsseoustissue,bonemarrow,connectivetissues,nerves,bloodvessels,fatAgenerallyacceptedclassificationsystemisbaseduponthepredominantmatrixcomponentandtypeofcelldifferentiationCLASSIFICATIONEachtypeoftissuegiverisetobenignormalignanttumours,butthedistinctionbetweenbenignandmalignanttumoursisnotalwaysclearCertaintumours,suchasosteoblastoma,giantcelltumour,andchondroblastomaoccasionallybehaveinanaggressivemannerSomemalignanttumourshavevariabledegreesofhistologicalaggressive:achondroscarcomamaybeclassifiedaslow,intermediate,orhighgradeThecharacterofthepainisusuallydullandaching.Alumporpathologicalfracturemayalsobefound.Anotableexceptionistheosteoidosteoma,painisdescribedassharporboring,worseatnight,andischaracteristiccompletelyrelievedwithaspirinPhysicalexaminationFindingsareminimalforbenignlesionsMalignantlesionsareoftenassociatedwithatendermassSomemalignanttumoursmaycauseheat,redness,oedema,venousdistension;thismakethedifferentiationfrominfectiondifficultSystemicfindingsarelackingexceptinpatientswithEwing'ssarcomaorlymphomawhomayshowfever,chills,andweightloss.LaboratoryTestsLaboratorytestsshouldincludeacompletebloodcountanderythrocytesedimentationratethesearehelpfulinexcludingdiseasessuchasmyeloma,leukaemia,andinfection.AlkalinephosphataseandlacticdehydrogenaselevelsareusefulintheassessmentofprognosisofosteosarcomaandEwing'ssarcoma.Serumimmunoelectrophoresisisdiagnosticformultiplemyeloma.ImagingStudiesRadiographRradionuclidebonescanCTMRIArteriogramsRadiographofabonelesionintwoplanesisthemostreliabletestThelesioncanbeassessedforitslocation,size,cortical,integrity,margination,andthepresenceorabsenceofasofttissuemassAchestradiographshouldbeobtainedinpatientwithasuspectedmalignantbonetumourtosearchformetastaticdiseaseAradionuclidebonescanisnotonlyinassessingtheactivityofthelesionbutalsoindeterminingthepresenceorabsenceofbonylesionsatothersitesMRIprovidesexcellentcontrastbetweenthetumourandsurroundingtissueandaidsindeterminingthemarrowandsofttissueextentCTismosthelpfulindefiningtheextentofbonedestructionbythetumourCTscansofthechestarealsoobtainedifamalignantbonetumourissuspecteddeterminingthemarrowandsofttissueextentArteriogramshavebeenusedlessfrequentlyfordiagnosissincetheadventofMRIbutareoccasionallyusefulifextremelyvascularlesionsneedtobeembolizedpreoperativelyTECHNIQUEOFBIOPSYAbiopsyspecimenisnecessarytoestablishthediagnosisAclosed(needle)biopsyOpenbiopsyAclosed(needle)biopsyofferstheadvantageofasmallpuncturewoundthatcanbeexcisedatthetimeoftheresectionNeedlebiopsyismostappropriateinlesionsofthespineorpelvisThediagnosticaccuracyisabout80percentOpenbiopsyisconsideredmorereliable,butithasthedisadvantagesofcreatingalargeroperativehaematomaPotentialforsofttissuecontaminationwithtumourcellsandincreasingthechanceofpathologicalfracturesifthecortexisviolatedSpecialimmunohistochemicalstainsandotherspecialtestshasbeenobtainedSURGICALSTAGINGImagingstudiesandbiopsyprovideinformationtoenabledefinitionofasurgicalstageforthelesionThemainvariablesindeterminingtheoutcomeforbonetumoursarethehistologicalgrade(G),theanatomicallocation(T),andthepresenceofdistantmetastases(M).GradingisbasedontumorsbiologicalbehaviourCertainlesions,suchasjuxtacorticalosteosarcomasisalwayslowgrade(G1)Thesurgicalsite(T)referstothelocationofthetumourIntracompartmentallesions(T1)aredefinedastumoursinwhichliewithinananatomicalcompartmentExtracompartmentallesions(T2)aredefinedastumoursinwhichthenaturalplanesaretransgressed,thetumourtissueinvolvesmorethanoneanatomicalcompartmentT1withincompartmentT2morethanonecompartmentThefinalmajorfactorindeterminingthesurgicalstageisthepresenceorabsenceofmetastases(M).ThelungsarethemostcommonsiteofspreadforsarcomasTheabsenceofmetastases,asdeterminedbychestCTandbonescan,resultsinadesignationofM0whilethepresenceofdistantspreadleadstoadesignationofM1Basedontheassessmentofsurgicalgrade(G),site(T),andmetastases(M),bonetumoursarestagedbythefollowingsystem:AllbenignbonetumoursareGOandStage0Lowgrademalignancies(G1)areStageI,whichisfurtherdividedintointracompartment(IA)andextracompartment(IB)Highgrademalignancies(G2)areclassifiedasStageIIAandIIBdependingontheintracompartmentorextracompartmentAlesionwithanyGorTwithdistantmetastasis(M)isconsideredStageIIIThisstagingsystemappearstobeaneffectiveaidinassessingprognosis,andprovidesabasisforsurgicalandothertreatmentsSURGICALPROCEDURESSurgicalresectionoramputationistheprimarymethodforlocalcontrolofmostbonetumours.ThesurgicalprocedureiscurrentlydescribedintermsofthestandarddefinitionswhichusinginanalysisandforcomparingresultsamongdifferentcentresSURGICALPROCEDURESThemargincanbeclassifiedas:Intra-lesional(theproceduretransectsorentersthelesion)Marginal(aplanethroughthereactivezoneor‘pseudocapsule'surroundingthetumor)Wide(leavingasignificantcuffofnormaltissuearoundthetumor)Radical(resectionofallanatomiccompartmentscontainingthetumour)Theintralesionalproceduresincludeopenbiopsyandcurettage,allofwhichleavemacroscopictumourbehindMarginalproceduresremovethebulkoftumourtissue,butarelikelytoleavesatelliteordaughternodulesWidemarginproceduresmayleavetheskiplesionsinthesurroundingnormaltissue.Rradicalmarginisremovedtheentirecompartment,andalllocaldiseaseisresumablyeradicatedIntra-lesional(transectingthelesion)Marginal(throughthereactivezoneorpseudocapsule)Wide(leavingasignificantcuff)Radical(resectingofallcompartment)BenignbonetumoursOsteoidosteomaCartilagetumoursEnchondromaChondroblastomaSolitaryosteocartilaginousexostosisFibrouscorticaldefectandnon-ossifyingfibromaFibrousdysplasiaSimpleorunicameralbonecystsAneurysmalbonecystOsteoidOsteomaBenignbonytumourofunknownaetiologyTheincidenceishighestintheseconddecade.ItismostcommonlylocatedinthetibiaandfemurTheclinicalpresentationiswithsharp,boringpainwhichisusuallyworseatnightandisoftenrelievedwithsalicylatesRadiographicallythelesionappearsasaradiolucent,roundorovalnidus.Thenidusisthelesionaltissueandisusually1cmorlessindiameter,butitissurroundedbyavariableamountofreactiveboneformationThediagnosisisapparentonCTorX-raySymptomsmaybetreatedwithsalicylatesMostlesionsarecuredbycompleteexcisionofthenidusradiolucent,roundorovalnidussurroundedbyavariableamountofreactiveboneformationBenigncartilagetumoursCartilaginoustumourscanbedividedintotwogroups:enostoticandexostoticlesionsEnostotictumourscompriseenchondroma,chondroblastoma,andchondromyxoidfibromaExostotictumoursconsistofsolitaryosteochondroma,juxtacorticalchondroma,andhereditarymultipleosteocartilaginousexostosisInradiographicalandhistologicalitmaybedifficulttodistinguishenchondromasfromchondrosarcomas,andchondroblastomaandchondromyxoidfibromaDistinguishingtheselesionsfromtheirmalignantcounterpartsisdifficult,butmalignantlesionsareunusualinthoselessthan30yearsMalignanttumoursarelargerthanbenigntumours,andareoftenpainfulandtenderEnchondromaBenigncartilagelesionlocatedcentrallywithinthemedullarycavityofshortandflatbonesTheyarethemostcommonbonetumourofthehandThelesionsmayoccuratanyageandareoftenasymptomaticunlessaccompaniedbyapathologicalfractureRadiographically,thelesionsarelucentandplacedcentrallywithinthemetaphysisordiaphysisoftheboneThereisusuallyamoderateamountofscleroticmarginationRoundedorstippledcalcificationisafrequentfindingTreatmentisprincipallybycurettageandpackingthedefectwithboneautograftsorallograftslucentandplacedcentrallyscleroticmarginationstippledcalcificationChondroblastomaMostcommoninadolescentUsuallyaffectstheepiphysisoflongbone.Theproximalanddistalfemur,proximalhumerusandproximaltibiaarethecommonsitesRadiographically,thelesionsareroundlucentandplacedcentrallywithintheepiphysis,characteristicstippledwithcalcificationsTreatmentisbycurettage,avoidingtheadjacentgrowthplateandarticularcartilageTherecurrencerateisabout25percentUnderrarecircumstances,benignchondroblastomasmayspreadtothelungslucentcentrallyplacewithintheepiphysis,stipplewithcalcificationsSolitaryosteocartilaginousexostosisfrequenttumourofboneTheconditionoccursatthemetaphysisofbonesatanyage.GrowthoftheselesionsceasesatmaturityRadiographically,exostosesappearassessileoutgrowthsofthemetaphysis,usuallyprojectingawayfromthejointsessileoutgrowthsofthemetaphysisprojectingawayfromthejointThepathologicaltissueisthecartilagecap.Theselesionsusuallyarenottroublesomeunlesstheycauseapainfulbursa,orinterferewithadjacentneurovascularstructuresMalignantdegenerationoccurswithanincidenceoflessthan0.1percentRecurrenceisunlikelyifthecartilagecapiscompletelyexcisedMultipleosteocartilaginousexostosisInheritedandischaracterizedbymultipleexostosiswhichcausedistortionoftheskeletonandmayleadtoseverefunctionalimpairmentThemaintreatmentisthemanagementofthedeformitiesinchildhoodandmonitoringthepossibledevelopmentofmalignancyFibrouscorticaldefect/non-ossifyingfibromaProbablynottrueneoplasmsOccurinthemetaphysesofthelongbonesinchildhoodEspeciallythedistalfemurandproximalanddistaltibiaMostoftheselesionsdisappearsoonafterlateadolescenceandgenerallyproducenosymptomsNotreatmentisrequiredunlessthelesionisindangercausingpathologicalfractureorthediagnosisisindoubt.Treatmentisprincipallybycurettageandbonegraft.FibrousdysplasiaDisorderoffibro-osseoustissueprobablynottrueneoplasmsRadiographically,thelesionshavealucentor‘groundglass'appearanceandcausethinningofthecortex.ThebonemaybeenlargedordeformedandmaybeinvolvedforitswholelengthTreatmentofthelesionsistopreventorcorrectofbonydeformitiesgroundglassappearanceenlargeordeformSimpleorunicameralbonecystslesionsofunknownaetiologymayoccurinthefirstdecadesThesecystsismostcommonlylocatedintheproximalhumerus(55percent)orproximalfemur(26percent)SimpleorunicameralbonecystsThecystappearsasacentral,radiolucentonthemetaphysisoflongbone.ThecortexisthinnedbutintactThelesionisusuallywelldemarcated.ThecavityisfilledwithafluidsimilartoserumTreatmentiscurettageandgraftingcentral,radiolucentonthemetaphysisoflongboneAneurysmalbonecystlesionofunknownaetiologyFoundinconjunctionwithlesionssuchasgiantcelltumour,chondroblastoma,chondromyxoidfibroma,orfibrousdysplasiaMostaneurysmalbonecystsoccurinpatientslessthan20yearsofageandaffectthelongbonesandspine.Radiographically,thelesionsareeccentricalexpansile,purelylytic,whicharequitewelldemarcatedfromtheboneandappearasa‘blowout'inthecorticalsurfacesurroundedbyaperiostealshellofnewboneeccentricalexpansile,purelylytic,welldemarcatedDifferentiationoftheselesionsfromtelangiectaticosteosarcomaandgiantcelltumourisimportantandoftendifficultTreatmentiscurettageandgraftingLesionsindifficultsitessuchasthepelvisorspineareoccasionallytreatedbyradiotherapyorembolizationGiantcelltumourAggressive,locallydestructivelesionofthemetaphyseo-epiphysealregionofthelongbonesThetumouroccursmostfrequentlybetweentheagesof20and40andismorecommoninwomenTheprincipalsitesofpredilectionarethedistalfemur,proximaltibia,proximalhumerusThetumourisusuallyeccentricallyplacedandlocatedinthemetaphysisandepiphysisofthelongbonesometimesextendingtothesubchondralcortexofthejointThecortexisalwaysthinnedThelesionisradiolucentandgivinganexpandedappearanceeccentricallyexpandedintheepiphysissoapbubblepatternThegiantcelltumourisdifficulttotreatThelocalrecurrenceratefollowingintralesionalsurgeryisover50percentTreatmentoptionsincludecurettageandpackingwithpolymethylmethacrylateorbonegraftWhenthelesionisextensive,aresectionandreconstructionwithallograftormetallicimplantsRadiotherapyisusuallysuccessfulineliminatingthetumour,butcarriestheriskofradiationnecrosisandradiation-inducedsarcoma.MalignantbonetumoursOsteosarcomaEnostotichyalinechondrosarcomasMalignantfibroustumoursFibrosarcomaMalignantfibroushistiocytomaChordomaEwing'ssarcomaMalignantbonetumoursMalignantbonetumoursvaryinbiologicalbehaviourfromlocalaggressivetumoursthatseldommetastasizewithapoorprognosis.Theyaffectallagegroupsandallbonysites.Diagnosisisoftendifficultandtreatmentiscomplicated.Osteosarcomamostcommonprimarymalignanttumourofbone,ischaracterizedbythedirectformationofosteoidtissuebystromaOsteosarcomaaccountingforabout40percentofmalignantbonetumorsTherearetwopeaksofincidence:thefirstoccursinpatientsbetween10and20years,itisaleadingcauseofcancermorbidityinadolescenceThesecondpeakoccursinthe50and60years;thosewhohavereceivedradiationtherapyOsteosarcomaismorecommoninmales,withasexratioof3:2.Themostcommonsitesoccurinthedistalfemoral,theproximaltibia,proximalhumerusThetumoursareusuallymetaphysealinlocation.Thecharacteristicsymptomsarepain,localtenderness,asofttissuemass,anddecreasedfunctionPhysicalexaminationdisclosesafirm,tendermassfixedtothesubjacentboneOccasionallythepatienthasapathologicalfracture.Therearenospecificlaboratoryfindings,theserumalkalinephosphataseandlacticdehydrogenaseconcentrationsareoftenincreasedRadiographicallythecortexisalwaystransgressed,andasofttissuemassiscommonTheperiosteumiselevatedandformreactiveboneattheperipheryofthelesion.Codman'striangle,Perpendicularstriations/sunburstappearanceOnion-skinningofperiosteum,arefrequentfindings.Codman'strianglesunburstappearancePerpendicularstriationsThelocationofthetumourappearstobeanimportantprognosticfactor:Proximalextremitylesionscarryaworseprognosisthandodistallesions,andlesionsoftheaxialskeletonhavetheworstprognosisThedurationofsymptoms,ageofthepatient,andthehistologicaltypeofosteosarcomaalsoinfluencesthefinaloutcomeInthepast,theoverall5-yearsurvivalrateforpatientswithosteosarcomatreatedbyamputationalonewas15to30percentCurrenttreatmentofosteosarcomaconsistsofcompletesurgicalexcisionandaggressiveadjuvantchemotherapy.Theoverall5-yearsurvivalrateis60to80percentMostpatientsreceivepreoperativechemotherapyfor2to3months,Followebywideorradicalexcisionofthetumour,andapproximately1yearofchemotherapy.Drugregimensincludemethotrexate,adriamycin,cytoxanandifosfamide.Neoadjuvantchemotherapyhastheadvantageofearliertreatmentofmicrometastaticdiseaseandmaymakelimbsalvagesurgerysaferandnearly80percentcanbetreatedbylimb-sparingoperationsTheuseofadjuvantchemotherapyhasimprovedthesurvivalofosteosarcomaLimb-sparingOperationsBoneallografts,Metallicprostheses,Arthrodeses,rRotationplasty,VascularizedbonetransfersandotheroptionsmaybeusedtoreconstructthebonydefectEnostotichyalinechondrosarcomasThelesionsarecentrallyplaced,metaphyseo-diaphysealinlocationThelesionmayproducethinningandcorticaltransgressionasofttissuemass,androundedorring-shapedcalcificationsCTisusuallyhelpfulinassessingtheextentandthedegreeofcorticaltransgressioncentrallyplacedthinningandcorticaltransgressionroundedorring-shapedcalcificationsTumoursusuallypresentwithpainandatendermassThetreatmentofchondrosarcomaofboneisalmostentirelysurgicalbuttherecurrencerateishighLimb-sparingprocedureswithwidemarginsareappropriateforlower-gradelesionsTreatmentofhigh-gradelesions,particularlywhenanextensivesofttissuemassispresent,mayrequireamputationAdjuvantchemotherapymaybeusedtocontrolmetastaticspreadofhigh-gradelesionsFibrosarcoma/Malignantfibroushistiocytomasimilarinage,clinical,andradiographicfeaturesoccurinallagegroupsandmostlyinlongbonesofthelowerextremitiesshowaslytic,poorlymarginated,metaphyseallesions,andareusuallyshowingcorticalbreakthrough,withantissuemassTreatmentislargelysurgicalLesionscanberesectedwithwidemarginsorradicalresectionoramputation.Radiotherapyandchemotherapyareoflittlebenefitandapproximatelyone-thirdofpatientssurvivelong-term.ChordomaisarareneoplasmarisefromnotochordalremnantsPatientsareusuallyinthe40tothe60yearsTheprincipalaffectedsegmentsarethesacrum.SacralchordomasareslowlygrowingandpatientsmaygivelonghistoriesofdiscomfortinthelowerspineTheneurologicalsymptomsincludeconstipation,sacralnervesensoryloss,weaknessofthemuscle,impotence,saddleanaesthesia,andfinally,lossofbladderandbowelcontrol.ChordomasarealwaysradiolucentandshowcorticaldestructtionandpoormarginationTheMRIisthemostusefulmeansofdefiningoftheselesionsinthesacrumregionCompleteexcisionoflargechordomasisdifficultPreoperativeradiationtherapyfollowedbylocalresectionmayreducetheincidenceoflocalrecurrenceEwing'ssarcomaisarare,round-celllesionaccountingforabout6percentofmalignantbonetumoursThetumourprincipallyoccursintheseconddecadeAnybonemaybeinvolved.Inthelongbones,thelesionshowsapredilectionformetaphysisbutitmayalsobediaphysisPain,swellingandintermittentfeversaretheusualfeaturesTheradiographicappearanceisthatofalyticdestructionandreactiveboneformationintheadjacentperiosteumTheperiostealreactiongivesan‘onionskinning’appearanceThefindingsarenon-specificandoftendifficulttodifferentiatefromosteosarcomaandinfection‘onionskinning’appearanceCurrently,surgeryhasusedtoresecttheprimarytumourincombinationwithradiationtherapyandchemotherapyThesurvivalratesisabout50to65percentEMPHASIS:SurgicalstagingSurgicalproceduresOsteosarcomaFURTHERREADINGTextbookofsurgeryOxfordtextbookofsurgeryEssentialofclinicalpracticePrinciplesofsurgeryTHANKYOU
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