软组织肿瘤分级与分期简介

软组织肿瘤的分级与分期简介,吴焕文,prognosis,PrognosisofSTSisdominatedbylocalrecurrenceanddistantmetastasis.Overallsurvivalmainlydependsonmetastasis,butinsomelocalizations,suchasretroperitonealareas,overallsurvivaldependsalsoonlocalrecurrence.Inmostreportedstudies,qualityofsurgicalmarginsisthemostimportantfactorforpredictinglocalrecurrence,whereasmetastasisandoverallsurvivalarerelatedmainlytohistologicgrade.,Histopathologicalgradingfavourableprognosisorpoorprognosis;overallsurvival;appropriatetreatmentregimenselection(Surgical,chemotherapeutic,andradiationtreatments),soft-tissuesarcomas,prognosticfactors,Gradingofsofttissuesarcomaswasfirstproposedin1939byBroders,whousedacombinationofmitoticactivity,tumorgiantcells,andfibrousstromainassigningagradetofibrosarcomas.Brodersalsoacknowledgedtheimportanceofcellulardifferentiationingrading.TherstcoherentandeffectiveprognosticclassicationofSTSwasproposedbyRusselletal.in1977.ThissystemintroducedahistologicgradingapplicabletoalladultSTSs,whichwasthemostimportantfactor.SomehistologicaltypeofTumorsaredefinitionallyhighgradeorlowgrade.Anumberofdifferentgradingsystemshavebeenproposedovertheyearsforsofttissuesarcomas,utilizing2tiered,3tiered,and4tieredstratificationschemes.FrenchFederationofCancerCenters(FNCLCC)system(37.3%)NationalCancerInstitute(NCI)(24%),Broderscriteria(12%)Markhedesystem(1.3%)other(15.3%),PARAMETERSUSEDINGRADING:HistologicaltypeNecrosisMitoticactivityTumourdifferentiationCellularityNuclearpleomorphismVascularinvasion,Themostwidelyusedandclinicallyvalidatedgradingsystemsare：NationalCancerInstitute(NCI)systemFrenchFederationofCancerCenters(FNCLCC)systemBothofwhichare3tieredsystems(Grade1,Grade2,Grade3).Atthepresenttime,theFNCLCCgradingsystemeaseofuseinterobserveragreementpredictivepower,NationalCancerInstitute(NCI)gradingsystem,Criteriaforgradinghistologicdiagnosiscellularitycellularpleomorphismmitoticratenecrosis：necrosisemergedasamajordiscriminatingvariable.grade2and3tumorsexhibitingmoderateormarkednecrosis(15%)hadasignificantlypoorerprognosis.Three-gradesystemTherespective5-yearsurvivalrates100%(GradeI)73%(GradeII)46%(GradeIII),NATIONALCANCERINSTITUTEGRADINGSYSTEM(NCI分级系统）Gradinginthissystemisbasedonhistologicaltypeoftumourandtumournecrosiswherehistologicaltypedoesnotdefinegrade.HISTOLOGICALPARAMETERGRADEHISTOLOGICALTYPE/SUBTYPE1-Epithelioidhaemangioendothelioma-Welldifferentiatedliposarcoma-Myxoidliposarcoma-InfantilefibrosarcomaHISTOLOGICALTYPE,MITOSIS,DIFFERENTIATION1-Well-differentiatedleiomyosarcoma(6mitosis/10HPF)-Well-differentiatedfibrosarcoma(6mitosis/10HPF)-Malignantperipheralnervesheathtumour(6mitosis/10HPF)-Extraskeletalmyxoidchondrosarcoma(nomitoses)HISTOLOGICALTYPE,NECROSIS2-Anysarcomanotcompulsorilygrade3andlessthan15%necrosisHISTOLOGICALTYPE/SUBTYPE3-Anysarcomawithmorethan15%necrosis-Rhabdomyosarcoma(allsubtypes)-Extraskeletalosteosarcoma-Ewingssarcoma/primitiveneuroectodermaltumour-Mesenchymalchondrosarcoma-Pleomorphicliposarcoma-Alveolarsoftpartsarcoma,ASSIGNEDHISTOLOGICGRADEACCORDINGTOHISTOLOGICTYPEINTHENCISYSTEM,Shortcomings,TheamountofnecrosisemphasizedbytheNCIsystemispotentiallyaffectedbypreferentialsamplingofnecroticandnon-necrotictissueNotpracticalforevaluationofneedlebiopsyspecimens.Moreover,retrospectiveanalysisiscomplicatedwhenanoverallassessmentofnecrosisisnotincludedinthegrossdescription.,FNCLCCgradingsystem,Criteriaforgradingcellulardifferentiationmitoticratetumornecrosis（镜下）reproducibility81%fortumornecrosis,74%fortumordifferentiation73%formitoticrate75%foroveralltumorgrade61%forhistologictypeTherespective5-yearsurvivalrates100%(GradeI)73%(GradeII)46%(GradeIII),Differentiationscoreisdefinedastheextenttowhichatumorresemblesadultmesenchymaltissue(score1)theextenttowhichthehistologictypeisknown(score2)theobservationthatthetumorisundifferentiated(score3),TUMORDIFFERENTIATIONSCOREACCORDINGTOHISTOLOGICTYPEINTHEUPDATEDVERSIONOFTHEFNCLCCSYSTEM,NCIVSFNCLCCgradediscrepancies：34.6%UseoftheFNCLCCsystemresultedin：anincreasednumberofgrade3tumorsareducednumberofgrade2tumorsabettercorrelationwithoverallandmetastasis-freesurvival,MIB-1system,Thehistologicalgradeisthemostusefulfactorforpredictingtheprognosisofsoft-tissuesarcomas.Amongothers,histologicalgradingonthebasisoftheMIB1labelingindexisadvantageous,bothintermsofobjectivityandreproducibility.,BothNCIandtheFNCLCCsystemsweredevelopedusingcohortsofpredominantlyadultpatients.Inchildren,gradingofsofttissuesarcomasiscompromisedbythegoodprognosisofcertaintumorssuchasinfantilefibrosarcoma.Inaddition,testingofagradingsystemwithinthepediatricpopulationisdifficultbecauseoftherarityoftheseneoplasms.Twosystemsarecurrentlyinuseforgradingpediatricnonrhabdomyosarcomatoussofttissuesarcoma(NRSTS)tumors.PediatricOncologyGroup(POG)gradingsystemsThegradingsystemsdevelopedbytheChildrensOncologyGroup(COG)andtheFrenchFederationofCancerCentersSarcomaGroup,Toavoidmodicationbytreatment,thehistologicalgradeshouldbeevaluatedinuntreatedprimarytumorspecimens.Sometumors(especiallypediatricrhabdomyosarcomas)mayappearmuchbetterdifferentiatedafterchemotherapy,but,intruth,thismostlikelyreflectsselectionofchemoresistantclonesoftumorcellsratherthananindicationofimprovedprognosis.Gradingsystembyanyothermeanscannotsubstitutefordistinctionbetweenbenignandmalignanttumorsordiagnosisofthehistologicaltypeofthetumors.Thesamegradingsystemcannotbeappliedtothedifferenttypesofsarcomasbecausetheprognosticfactorsdifferaccordingtohistologictype.Thehistologicalgradedoesnotserveasareliableprognosticfactorformalignantperipheralnervesheathtumors(MPNST)andtumorsthatoftendevelopinchildren.Itisalsonotusefulincasesofalveolarsoft-partsarcoma,clearcellsarcomaorepithelioidsarcomaasthesesarcomasshowhistiotype-specicbehavior.extraskeletalmyxoidchondrosarcoma,“Low-grade”fibromyxoidsarcomaCutaneousangiosarcoma：thesizeoftheprimarytumorSynovialsarcoma：年龄、大小、是否存在低分化区域分为高危组和低危组EwingsarcomashouldbeconsideredautomaticallyasaGrade3sarcomawithahighmetastaticpotential.Totalkaboutdegreeofdifferentiationintumorsthathavenodifferentiatednormaltissueequivalent,suchassynovialsarcoma,epithelioidsarcoma,andalveolarsoftpartsarcoma,isclearlypointless.,Histologicalgradingisusefulforpredictingthelikelihoodofdistantmetastasesandprognosisinagivenpatient.Itislessusefulforpredictingthelikelihoodoflocalrecurrence,whichisknowntobemorecloselyassociatedwiththemarginofresection.Furthermore,specimensxedwellandobtainedfromatumor-richareashouldbeusedforthisclassication.appropriatelyxedandprocessedspecimensmustbeused;themitoticguresmustbecheckedaccurately;Themitoticrateineachvisualeldiscountedatamagnicationlevelof400,andthecountsin10visualeldsaretotaledtoyieldthenumberofmitoticgures(per10high-powerelds,HPF).anareaofthespecimenwithahighcellularityandtheapparentlylargestnumberofmitoticguresmustbeselected.Theseprecautionsarethesameasthosecommonlyapplicabletomeasurementofthemitoticcountinpathologicalspecimens.Needlebiopsiesarenotidealforpreoperativegradingpurposes.Ingastrointestinalstromaltumor,theNIHconsensusriskassessmentsreportswereusedasasurrogateforhistologicgrade.,Accuratehistologicaltypingofthetumourplaysanimportantroleindeterminingtumourprognosisinthefollowingcases:HIGHGRADETUMOURSAngiosarcomaExtraskeletalEwingssarcoma/PNETExtraskeletalosteosarcomaroundcellliposarcomaMesenchymalchondrosarcomaPleomorphicliposarcomaRhabdomyosarcomaLOWGRADETUMOURSAngiomatoidmalignantfibroushistiocytomaAtypicalfibroxanthomaAtypicallipomatoustumour/welldifferentiatedliposarcomaDermatofibrosarcomaprotuberansDesmoidtumourMyxoidliposarcoma,Thespectrumofgradesobservedamonghistologicsubtypesofsoft-tissuesarcoma.(FromEnzingerFNandWeissSW,editors.SoftTissueTumors.）,STS的分期,UICC/AJCCTNM分期PRIMARYTUMOR(T)REGIONALLYMPHNODES(N)DISTANTMETASTASIS(M),ThecurrentAJCCSTSstagingsystem：Unlikewithotherorgansystems,thestagingofsofttissuesarcomasislargelydeterminedbygrade.fourcriteriaoftumorsize(depth),nodalstatus,grade,andmetastasis(TNGM).usefulprognosticinformationselectionofpatientsforadjuvanttherapyand/orstraticationforinclusioninclinicaltrials.,PastEditionsoftheAJCCCancerStagingManualEdition1published1977andwentintoeffect1978Edition2published1983andwentintoeffect1984Edition3published1988andwentintoeffect1989Edition4published1992andwentintoeffect1993Edition5published1997andwentintoeffect1998Edition6published2002andwentintoeffect2003Edition7published2009andwentintoeffect2010,Depth：Besideshistologicgrade,tumordepth(superficialversusdeep)wasanotherimportantprognosticparameter.,TheTNMstagingsystemforsofttissuetumorsoftheAJCCandUICCisrecommended.Thestagingsystemappliestoallsofttissuesarcomas,exceptKaposisarcomagastrointestinalstromaltumorsfibromatosis(desmoidtumor)infantilefibrosarcoma.notoptimallystagedbythissystemsarcomasarisingwithintheconfinesoftheduramater,includingthebrainsarcomasarisinginparenchymatousorgansandfromhollowviscera,AnatomicStage/PrognosticGroupsAJCCCancerStagingManual.7thed.,AnatomicStage/PrognosticGroupsAJCCCancerStagingManual.6thed.,Nodalinvolvementisrare,occurringinlessthan5%ofpatientswithsarcoma.Histologicsubtypesofsofttissuesarcoma,suchasrhabdomyosarcomaandepithelioidsarcoma,havethegreatestpredilectionforlymphnodeinvolvement.,Patientswithisolatedlymphnodemetastasishadanestimated4-yearOSrateof71%,whichwasequivalenttothesurvivalofAJCCstageIIIpatients.Incontrast,thosepatientswhohadsynchronoussystemicandlymphnodeinvolvementhadfarworseoutcomesthatwerecomparabletothoseofstageIV.ThesesuggestthatisolatedlymphnodemetastasesaremostlikelytobeassociatedwithanAJCCstageIIIratherthanstageIVsurvivalpattern,Enneking分期肌肉骨骼肌协会分期系统usedprimarilybyorthopediconcologists,Enneking分期,两种解剖学情况：T1：间室内，是指局限于容易辨认的解剖学结构（如功能性肌群、关节和皮下组织）T2：间室外，指来源于或继发性侵犯无自然解剖屏障区域的肿瘤两级(G1，G2)，其与外科手术的两种治疗方式（扩大切除、根治切除）相吻合。三期（IA/B,IIA/B,III)适用软组织肉瘤及骨肉瘤，最适用于四肢病变。,Thesurgicalgrademaydifferslightlyfromthepurelyhistologicgradebyconsiderationofclinicalandradiographicfeatures.,Stagingofrhabdomyosarcoma,Theprocessincludesthefollowingsteps:Assigningastage(considersite,size,Surgico-pathologicGroup,andpresence/absenceofmetastases).AssigningalocaltumorSurgico-pathologicGroup(statuspostsurgicalresection/biopsy,withpathologicassessmentofthetumormargin).AssigningaRiskGroup(classifiedbyStage,Group,andhistology).,SoftTissueSarcomaCommitteeoftheChildrensOncologyGroupPretreatmentStagingSystem,SoftTissueSarcomaCommitteeoftheChildrensOncologyGroupSurgico-pathologicGroupSystem,SoftTissueSarcomaCommitteeoftheChildrensOncologyGroupRhabdomyosarcomaRiskGroupClassification,PrognosticparametersnotcurrentlyincludedinthepresentAJCCSTSstagingsystem,AgeAgewasthemostconsistentadverseindependentprognosticfactorforsurvival.Neurovascularandboneinvasionindependentpredictors:malignantbroushistiocytomas;leiomyosarcomas;synovialsarcoma;,Histologicsubtypechildrenandadolescentsnon-rhabdomyosarcomasofttissuesarcoma(NRSTS):lessresponsivetoradiotherapyrhabdomyosarcoma:radiotherapycanbeusedinsteadofsurgeryembryonalsubtypesalveolarsubtypesretroperitonealsarcomas(RPSs)atypicallipomatoustumors(ALTs),non-ALTliposarcomas(LPSs)other,Siteofprimarydisease：,SpecicmolecularprognosticmarkerslocalrecurrenceMostanalysesidentifypreviouslocalrecurrenceasamajor(ifnotthemajor)riskfactorforsubsequentlocalrecurrence.,MarginstatusPositivesurgicalmarginsarethemainpredictorsforlocalrelapse.Grade,size,andTNMstage(UICC/AJCC)havestrongerprognosticsignicanceforoverallsurvivalanddistantrecurrencethanforlocalrelapse.,Itisbelievedgenerallythatsurgicalmarginsoflessthan1.5-2cminsofttissuesarcomapredisposetoanincreasedriskoflocalrecurrenceunlessfurthersurgeryorirradiationisundertaken.Howeverifasurgicalmarginisboundedbyanunbreachedlayeroffasciaorperiosteumthisriskprobablydoesnotapply,butsuchmarginsshouldstillbemeasuredifclose.,MultivariateAnalysisofPrognosticFactorsinPatientswithExtremitySoft-TissueSarcoma,RECOMMENDATIONSFORTHEREPORTINGOFSOFTTISSUESARCOMADiagnosticInformation1.Siteanddepthoftumor(e.g.dermal,subcutaneous,fascial,subfascial,intramuscular,visceralormorethanoneofthese).2.Histologicaltype(useWHOsystemwhenpossible);iftumortypeisunknownthenthetermunclassifiedsarcomawithaqualifiersuchaspleomorphic,spindlecell,myxoidorroundcellisuseful.3.Maximaldimensionoftumor(incm);4.Histologicgrade;5.Minimumdistance(s)toresectionmargins-anymarginlessthan2cmfromthetumorshouldbespecifiedintermsoflocationanddistance;,6.Histologicevidenceofapre-existingbenignlesion(onlyapplicabletonervesheathneoplasms);7.Lymphnodestatus(ifpresent);8.Resultsofanyspecialinvestigations(e.g.specialstains,immunohistochemistry,electronmicroscopy,DNAflowcytometry,karyotype).,1.Mitoticrate,expressedasnumberofmitosesper10highpowerfields;2.Extentofnecrosis,asconfirmedhistologically;3.Presenceorabsenceofvascularinvasion,irrespectiveofvesseltype;4.Characteroflesi