软骨肉瘤.pptx

Chondrosarcoma 软骨肉瘤胸组病例讨论09.11.10DiaphysisMyeloid多发性骨髓瘤（Multiple myeloma）Epithelial釉质细胞瘤（Adamantinoma）PNETEwings sarcomaMetaphysisOsteoblast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（Ossifying fibroma）纤维肉瘤（Fibrosarcoma）EpiphysisChondroblast成软骨细胞瘤（Chondroblastoma）Myeloid骨巨细胞瘤（Giant cell tumor of bone）OtherNotochord脊索瘤(Chordoma)SITE OF LONG BONE INVOLVEMENT(most primary bone tumors have favored sites within long bones;this may provide a clue to diagnosis).Diaphyseal intramedullary lesions:Favored location for Ewings sarcoma,lymphoma,myeloma.Common for fibrous dysplasia and enchondroma Metaphyseal lesions centered in the cortex:Classic location for a non-ossifying fibroma(NOF).Also,a common site for osteoid osteoma.Epiphyseal lesions:Chondroblastoma(Ch)and Giant Cell Tumor(GCT)are almost invariably centered in the epiphysis.Metaphyseal exostosis:OsteochondromaMetaphyseal intramedullary lesions:Osteosarcoma is usually centered in themetaphysis.Chondro-sarcoma and fibrosarcoma often present as metaphyseal lesions.Osteoblastoma,enchondroma,fibrous dysplasia,simple bone cyst,and aneurysmal bone cyst are commonin this location.Diaphyseal lesions centered in the cortex:Adamantinoma,osteoid osteoma AGE(probably the most important clinical clue).Age group Most common benign lesions Most common malignant tumors 0-10 simple bone cysteosinophilic granuloma(嗜酸性肉芽肿)Ewings sarcomaleukemic involvementmetastatic neuroblastoma 10-20 non-ossifying fibromafibrous dysplasiasimple bone cystaneurysmal bone cystosteochondroma(exostosis)osteoid osteomaosteoblastomachondroblastomachondromyxoid fibroma osteosarcoma,Ewings sarcoma,adamantinoma 20-40 enchondroma giant cell tumor chondrosarcoma 40&above osteoma metastatic tumorsmyelomaleukemic involvementchondrosarcomaosteosarcoma(Pagets associated)MFHchordoma IntroductionChondrosarcomas are malignant neoplasms populated by mesenchymal cells that produce a cartilaginous matrix.3.5%of all primary bone tumors and 20%-27%of primary malignant osseous neoplasms,exceeded in frequency only by multiple myeloma and osteosarcoma.Most patients are in the 40-60 year range with a 2:1 male predilectionIntroductionThe bones of the shoulder,pelvis,proximal femur and ribs are most commonly involvedIn contrast to enchondroma,chondrosarcoma rarely involves the distal extremitiesIntroductionCategories of primary chondrosarcomas have been described,including：conventional intramedullary（普通髓内型）,juxtacortical（皮质旁型）,extraskeletal（骨外型）,clear cell（透明细胞型）,myxoid（粘液型）,mesenchymal（间质型）and dedifferentiated（去分化型）.IntroductionChondrosarcomas present most often as enlarging,painful masses;their growth rate and ultimate behavior is closely correlated with histologic grade.Chondrosarcomas metastasize via the hematogenous route,most often to the lungs.5-year survival rate was 90%,81%and 43%in grades 1 through 3 in one study.The aggressiveness of chondrosarcomas can be predicted by their histologic grade.Grading system is based on three parameters:cellularity,degree of nuclear atypia and mitotic activity.Grade 1(low-grade)Very similar to enchondroma.However,the cellularity is higher,and there is mild cellular pleomorphism.The nuclei are small but often show open chromatin pattern and small nucleoli.Binucleated cells are frequent.Mitoses are very rare.Grade 1 chondrosarcomas are locally aggressive and prone to recurrences,but usually do not metastasize.Grade 2(low-grade)The cellularity is higher than in Grade 1 tumors.Characteristic findings are moderate cellular pleomorphism,plump nuclei,frequent bi-nucleated cells,and occasional bizarre cells.Mitoses are rare.Foci of myxoid change may be seen.Unlike Grade 1 tumors,about 10%to 15%of Grade 2 chondrosarcomas produce metastases.Grade 3(high-grade)Characteristic findings are high cellularity,marked cellular pleomorphism,high N/C ratio,many bizarre cells and frequent mitoses(more than 1 per hpf).These are high grade tumors with significant metastatic potential.21-year-old man with shoulder pain.60-year-old man30-year-old man with hip pain37-year-old man with a painful lump in his right leg for 1 yearConclusionsAlthough the pathologic appearance varies with specific lesion type,chondrosarcomas grow with lobular type architecture,and these hyaline cartilage nodules demonstrate high water content and peripheral enchondral ossification.