软骨肉瘤

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,#,Chondrosarcoma,软骨肉瘤,胸组病例讨论,09.11.10,Diaphysis,Myeloid,多发性骨髓瘤（,Multiple myeloma,）,Epithelial,釉质细胞瘤（,Adamantinoma,）,PNET,Ewings sarcoma,Metaphysis,Osteoblast,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,Chondroblast,成软骨细胞瘤（,Chondroblastoma,）,Myeloid,骨巨细胞瘤（,Giant cell tumor of bone,）,Other,Notochord,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:,Osteochondroma,Metaphyseal intramedullary,lesions:,Osteosarcoma is,usually centered in the,metaphysis.,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 involvement,chondrosarcoma,osteosarcoma(Pagets associated)MFHchordoma,Introduction,Chondrosarcomas 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 predilection,Introduction,The bones of the shoulder,pelvis,proximal femur and ribs are most commonly involved,In contrast to enchondroma,chondrosarcoma rarely involves the distal extremities,Introduction,Categories 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）,.,Introduction,Chondrosarcomas 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 man,30-year-old man with hip pain,37-year-old man with a painful lump in his right leg for 1 year,Conclusions,Although the pathologic appearance varies with specic lesion type,chondrosarcomas grow with,lobular type architecture,and these hyaline cartilage nodules demonstrate,high water content,and,peripheral enchondral ossication,.,谢谢！,