软骨肉瘤的学习资料

软骨肉瘤的学习资料第1页/共16页DiaphysisMyeloid多发性骨髓瘤（Multiplemyeloma）Epithelial釉质细胞瘤（Adamantinoma）PNETEwing’ssarcomaMetaphysisOsteoblast骨样骨瘤成骨细胞瘤（Osteoblastoma）骨瘤（Osteoma）骨肉瘤（Osteosarcoma）Chondroblast软骨瘤软骨肉瘤（Chondrosarcoma）骨软骨瘤软骨粘液样纤维瘤Fibrous骨化纤维瘤（Ossifyingfibroma）纤维肉瘤（Fibrosarcoma）EpiphysisChondroblast成软骨细胞瘤（Chondroblastoma）Myeloid骨巨细胞瘤（Giantcelltumorofbone）OtherNotochord脊索瘤(Chordoma)第2页/共16页SITEOFLONGBONEINVOLVEMENT

(mostprimarybonetumorshavefavoredsiteswithinlongbones;thismayprovideacluetodiagnosis).

Diaphysealintramedullarylesions:

FavoredlocationforEwing'ssarcoma,lymphoma,myeloma.CommonforfibrousdysplasiaandenchondromaMetaphyseallesionscenteredinthecortex:

Classiclocationforanon-ossifyingfibroma(NOF).Also,acommonsiteforosteoidosteoma.

Epiphyseallesions:

Chondroblastoma(Ch)andGiantCellTumor(GCT)arealmostinvariablycenteredintheepiphysis.

Metaphysealexostosis:

Osteochondroma

Metaphysealintramedullarylesions:

Osteosarcomaisusuallycenteredinthemetaphysis.Chondro-sarcomaandfibrosarcomaoftenpresentasmetaphyseallesions.Osteoblastoma,enchondroma,fibrousdysplasia,simplebonecyst,andaneurysmalbonecystarecommoninthislocation.Diaphyseallesionscenteredinthecortex:

Adamantinoma,osteoidosteoma

第3页/共16页AGE(probablythemostimportantclinicalclue).

Agegroup

Mostcommonbenignlesions

Mostcommonmalignanttumors

0-10

simplebonecyst

eosinophilicgranuloma(嗜酸性肉芽肿)

Ewing'ssarcoma

leukemicinvolvement

metastaticneuroblastoma10-20non-ossifyingfibroma

fibrousdysplasia

simplebonecyst

aneurysmalbonecyst

osteochondroma(exostosis)

osteoidosteoma

osteoblastoma

chondroblastoma

chondromyxoidfibromaosteosarcoma,

Ewing'ssarcoma,

adamantinoma20-40

enchondroma

giantcelltumor

chondrosarcoma

40&aboveosteomametastatictumors

myeloma

leukemicinvolvement

chondrosarcoma

osteosarcoma(Paget'sassociated)

MFH

chordoma第4页/共16页IntroductionChondrosarcomasaremalignantneoplasmspopulatedbymesenchymalcellsthatproduceacartilaginousmatrix.3.5%ofallprimarybonetumorsand20%-27%ofprimarymalignantosseousneoplasms,exceededinfrequencyonlybymultiplemyelomaandosteosarcoma.Mostpatientsareinthe40-60yearrangewitha2:1malepredilection第5页/共16页IntroductionThebonesoftheshoulder,pelvis,proximalfemurandribsaremostcommonlyinvolvedIncontrasttoenchondroma,chondrosarcomararelyinvolvesthedistalextremities第6页/共16页IntroductionCategoriesofprimarychondrosarcomashavebeendescribed,including：

conventionalintramedullary（普通髓内型）,juxtacortical（皮质旁型）,extraskeletal（骨外型）,clearcell（透明细胞型）,myxoid（粘液型）,mesenchymal（间质型）anddedifferentiated（去分化型）.第7页/共16页IntroductionChondrosarcomaspresentmostoftenasenlarging,painfulmasses;theirgrowthrateandultimatebehavioriscloselycorrelatedwithhistologicgrade.Chondrosarcomasmetastasizeviathehematogenousroute,mostoftentothelungs.5-yearsurvivalratewas90%,81%and43%ingrades1through3inonestudy.第8页/共16页Theaggressivenessofchondrosarcomascanbepredictedbytheirhistologicgrade.Gradingsystemisbasedonthreeparameters:cellularity,degreeofnuclearatypiaandmitoticactivity.Grade1(low-grade)

Verysimilartoenchondroma.However,thecellularityishigher,andthereismildcellularpleomorphism.Thenucleiaresmallbutoftenshowopenchromatinpatternandsmallnucleoli.Binucleatedcellsarefrequent.Mitosesareveryrare.Grade1chondrosarcomasarelocallyaggressiveandpronetorecurrences,butusuallydonotmetastasize.Grade2(low-grade)

ThecellularityishigherthaninGrade1tumors.Characteristicfindingsaremoderatecellularpleomorphism,plumpnuclei,frequentbi-nucleatedcells,andoccasionalbizarrecells.Mitosesarerare.Fociofmyxoidchangemaybeseen.UnlikeGrade1tumors,about10%to15%ofGrade2chondrosarcomasproducemetastases.Grade3(high-grade)

Characteristicfindingsarehighcellularity,markedcellularpleomorphism,highN/Cratio,manybizarrecellsandfrequentmitoses(morethan1perhpf).Thesearehighgradetumorswithsignificantmetastaticpotential.第9页/共16页

21-year-oldmanwithshoulderpain.第10页/共16页60-year-oldman第11页/共16页30-year-oldmanwithhippain第12页/共16页37-year-oldmanwithapainfullumpinhisrightlegfor1year第13页/共16页ConclusionsAlthoughthepathologicappearancevarieswit