课件：软骨肉瘤.ppt

Chondrosarcoma 软骨肉瘤,胸组病例讨论 09.11.10,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,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 speci