软组织肿瘤的分级与分期简介吴焕文prognosisPrognosisofSTSisdominatedbylocalrecurrenceanddistantmetastasis.Overallsurvivalmainlydependsonmetastasis,butinsomelocalizations,suchasretroperitonealareas,overallsurvivaldependsalsoonlocalrecurrence.Inmostreportedstudies,qualityofsurgicalmarginsisthemostimportantfactorforpredictinglocalrecurrence,whereasmetastasisandoverallsurvivalarerelatedmainlytohistologicgrade.Histopathologicalgradingfavourableprognosisorpoorprognosis;overallsurvival;appropriatetreatmentregimenselection(Surgical,chemotherapeutic,andradiationtreatments)soft-tissuesarcomasprognosticfactorsGradingofsofttissuesarcomaswasfirstproposedin1939byBroders,whousedacombinationofmitoticactivity,tumorgiantcells,andfibrousstromainassigningagradetofibrosarcomas.Brodersalsoacknowledgedtheimportanceofcellulardifferentiationingrading.ThefirstcoherentandeffectiveprognosticclassificationofSTSwasproposedbyRusselletal.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)system–FrenchFederationofCancerCenters(FNCLCC)system•Bothofwhichare3tieredsystems(Grade1,Grade2,Grade3).•Atthepresenttime,theFNCLCCgradingsystemeaseofuseinterobserveragreementpredictivepowerNationalCancerInstitute(NCI)gradingsystemCriteriaforgrading•histologicdiagnosiscellularity•cellularpleomorphism•mitoticrate•necrosis: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-Ewing'ssarcoma/primitiveneuroectodermaltumour-Mesenchymalchondrosarcoma-Pleomorphicliposarcoma-AlveolarsoftpartsarcomaASSIGNEDHISTOLOGICGRADEACCORDINGTOHISTOLOGICTYPEINTHENCISYSTEMHistologictypeGrade1Grade2Grade3Well-differentiatedliposarcoma+Myxoidliposarcoma+Roundcellliposarcoma++Pleomorphicliposarcoma+Fibrosarcoma++MFH,pleomorphictype++MFH,inflammatorytype++MFH,myxoidtype+MFH,pleomorphictype+DFSP+Malignantgranularcelltumor++Leiomyosarcoma+++Malignanthemangiopericytoma+++Rhabdomyosarcoma(alltypes)+Chondrosarcoma+++Myxoidchondrosarcoma++Mesenchymalchondrosarcoma+Osteosarcoma+ExtraskeletalEwingsarcoma+Synovialsarcoma+Epithelioidsarcoma++Clearcellsarcoma++SuperficialMPNST+EpithelioidMPNST++MalignantTritontumor+Angiosarcoma++Alveolarsoftpartsarcoma+Kaposisarcoma++Shortcomings•TheamountofnecrosisemphasizedbytheNCIsystemispotentiallyaffectedbypreferentialsamplingofnecroticandnon-necrotictissue•Notpracticalforevaluationofneedlebiopsyspecimens.•Moreover,retrospectiveanalysisiscomplicatedwhenanoverallassessmentofnecrosisisnotincludedinthegrossdescription.FNCLCCgradingsystemCriteriaforgradingcellulardifferentiationmitoticratetumornecrosis(镜下)reproducibility81%fortumornecrosis,74%fortumordifferentiation73%formitoticrate75%foroveralltumorgrade61%forhistologictypeTherespective5-yearsurvivalrates100%(GradeI)73%(GradeII)46%(GradeIII)Differentiationscoreisdefinedastheextenttowhichatumorresemblesadultmesenchymaltissue(score1)theextenttowhichthehistologictypeisknown(score2)theobservationthatthetumorisundifferentiated(score3)TUMORDIFFERENTIATIONSCOREACCORDINGTOHISTOLOGICTYPEINTHEUPDATEDVERSIONOFTHEFNCLCCSYSTEMHistologictypescoreWell-differentiatedliposarcoma1Myxoidliposarcoma2Roundcellliposarcoma3Pleomorphicliposarcoma3Dedifferentiatedliposarcoma3Well-differentiatedfibrosarcoma1Conventionalfibrosarcoma2Poorlydifferentiatedfibrosarcoma3Well-differentiatedMPNST1ConventionalMPNST2PoorlydifferentiatedMPNST3EpithelioidMPNST3Malignanttritontumor3Well-differentiatedmalighemangiopericytoma2Conventionalmalighemangiopericytoma3MyxoidMFH2Typicalstoriform/pleomorphicMFH2Giant-cellandinflammatoryMFH3Well-differentiate