软骨肉瘤.ppt

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1、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）Epiphysis

2、Chondroblast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,

3、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 epiph

4、ysis.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

5、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 neuroblasto

6、ma 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 tumorsmy

7、elomaleukemic 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 freque

8、ncy 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 extremitiesIntroduct

9、ionCategories 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 g

10、rowth 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 histol

11、ogic 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 smal

12、l 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 n

13、uclei,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 r

14、atio,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.