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Osteosarcoma

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  • 1.  Definition Introduction and brief history Epidemiology Etiology Pathology Classification
  • 2.  A mesenchymalmalignancy thatdifferentiates toproduce osteoid is anosteosarcoma nomatter how muchosteoid is produced OsteoidDeposition
  • 3.  Malignant tumor ofmesenchymal originhaving Spindle shapedcells that produce osteoid Second most commonprimary malignancy ofbone 15% of all biopsiedprimary bone tumors
  • 4.  In 1805, the French surgeon Alexis Boyer firstused the term "osteosarcoma’’ In 1847, the Baron Guillaume Dupuytren describedgross pathologic appearance of osteosarcoma Jaffe and Lichtenstein established histologiccriteria to diagnose common bone tumors
  • 5.  In the 1970s, Dr Norman Jaffe used variety ofeffective chemotherapeutic agents Dr William F. Enneking  introduced his surgicalstaging system for musculoskeletal sarcomas.
  • 6.  In the United States, the incidence ofosteosarcoma is 400 cases per year (4.8 permillion population <20 y).higher in blacks than in whiteshigher in males than in females The overall 5-year survival rate is 63%
  • 7.  Sites:Metaphysis: 90%Diaphysis: 8-10%- can occur in any boneLong Bones: 70%-80%Distal Femur (40%)Proximal Tibia (20%)Proximal Humerus(15%)Axial SkeletonPelvis
  • 8.  The exact cause of osteosarcoma is unknownRisk factors – Rapid bone growth Environmental Genetic Pre-existing benign /malignant lesions
  • 9.  Rapid bone growth- increased incidence during theadolescent growth spurt- location in the metaphyseal area adjacent to thegrowth plate (physis) of long bones GeneticLi-Fraumeni syndrome (p53 mutation)Rothmund-Thomson syndromeMutation of the RB gene (retinoblastoma)
  • 10.  EnvironmentalRadiation – localised radiation >2000 radslatent period -4 years to 40 yearsalpha rays>beta raysChemicals – methylcholanthreneacetylaminofluoreneberyllium compoundsVirus - RNA :mouse sarcoma virusDNA :polyoma ,SV 40 virus
  • 11.  Paget disease fibrous dysplasia enchondromatosis hereditary multipleexostoses chondrosarcoma
  • 12.  Gross appearanceSclerosing or osteolyticTypical featuresmetaphyseal locationvariable consistency andcoloursubperiosteal spreadperiosteum,epiphysis,cartilage act as barrier
  • 13. Gross appearance Greyish white colour Consistency variesfromSoft and gritty to hard Erodes inner cortex Invades subperiostealspace Penetrate periosteumand invades soft tissue
  • 14. Microscopic features Extensive irregularsheets of new osteoid Peripheral hypercellularstroma with anaplasticcells forming osteoid Central cells entrapedby osseous tissuebecome small androunded
  • 15. Gross appearance Very vascular tumour Consistency soft andfriable Necrotic andhemorrhagic cavitiesinterspersed withfibrous tissue Pathological fracturecommon
  • 16. Microscopic features Blood containingspaces withoutendothelial lining Sparse osseous tissue Spindle cells,anaplastic osteoblast,giant cells are seen
  • 17.  Anatomic location Degree of differentiation Multicentricity Etiology Histology
  • 18.  Intramedullary (75%) Juxtacortical/Surface (7--10%) Intracortical (.2%)
  • 19.  conventionalOsteoblasticChondroblasticFibroblasticSmall-cellEpithelioid Telangiectatic Well--differentiated
  • 20. Juxtacortical Osteosarcoma Parosteal Osteosarcoma Periosteal Osteosarcoma High Grade Surface Osteosarcoma
  • 21.  Origin: Arises fromouter layer ofperiosteum Usually a low gradetumor with fibroblasticstroma andosteoid/woven bone
  • 22.  Origin: Arises fromtheinner layer of theperiosteum Low to intermediategrade bone withpredominantchondroblasticdifferentiation
  • 23. Parosteal Osteosarcoma Periosteal Osteosarcoma
  • 24.  High Grade Surfaceosteosarcoma
  • 25.  Very rare High grade confined to the cortex Sites: Diaphysis offemur or tibia
  • 26.  Synchronous Osteosarcoma:Lesions that affect multiple bonesdiscovered within 6 months of each other Metachronous Osteosarcoma:Lesions involving multiple bonesdiscovered more than 6 months apart
  • 27.  Malignant transformation of benign conditionPaget diseasefibrous dysplasiabone infarct Arising in dedifferentiated chondrosarcoma
  • 28.  A mesenchymal malignancy that differentiates toproduce osteoid bone Second most common primary malignant tumor ofbone Site - metaphysis of long bone Age – adolescent (15 – 25 years)
  • 29.  Etiology not known Associated risk factors –rapid bone growth in youngpre-existing bone lesion in old age Most common - Conventional Osteosarcoma
  • 30. Thank you

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