Osteosarcoma

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Osteosarcoma

  1. 1.  Definition Introduction and brief history Epidemiology Etiology Pathology Classification
  2. 2.  A mesenchymalmalignancy thatdifferentiates toproduce osteoid is anosteosarcoma nomatter how muchosteoid is produced OsteoidDeposition
  3. 3.  Malignant tumor ofmesenchymal originhaving Spindle shapedcells that produce osteoid Second most commonprimary malignancy ofbone 15% of all biopsiedprimary bone tumors
  4. 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. 5.  In the 1970s, Dr Norman Jaffe used variety ofeffective chemotherapeutic agents Dr William F. Enneking  introduced his surgicalstaging system for musculoskeletal sarcomas.
  6. 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. 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. 8.  The exact cause of osteosarcoma is unknownRisk factors – Rapid bone growth Environmental Genetic Pre-existing benign /malignant lesions
  9. 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. 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. 11.  Paget disease fibrous dysplasia enchondromatosis hereditary multipleexostoses chondrosarcoma
  12. 12.  Gross appearanceSclerosing or osteolyticTypical featuresmetaphyseal locationvariable consistency andcoloursubperiosteal spreadperiosteum,epiphysis,cartilage act as barrier
  13. 13. Gross appearance Greyish white colour Consistency variesfromSoft and gritty to hard Erodes inner cortex Invades subperiostealspace Penetrate periosteumand invades soft tissue
  14. 14. Microscopic features Extensive irregularsheets of new osteoid Peripheral hypercellularstroma with anaplasticcells forming osteoid Central cells entrapedby osseous tissuebecome small androunded
  15. 15. Gross appearance Very vascular tumour Consistency soft andfriable Necrotic andhemorrhagic cavitiesinterspersed withfibrous tissue Pathological fracturecommon
  16. 16. Microscopic features Blood containingspaces withoutendothelial lining Sparse osseous tissue Spindle cells,anaplastic osteoblast,giant cells are seen
  17. 17.  Anatomic location Degree of differentiation Multicentricity Etiology Histology
  18. 18.  Intramedullary (75%) Juxtacortical/Surface (7--10%) Intracortical (.2%)
  19. 19.  conventionalOsteoblasticChondroblasticFibroblasticSmall-cellEpithelioid Telangiectatic Well--differentiated
  20. 20. Juxtacortical Osteosarcoma Parosteal Osteosarcoma Periosteal Osteosarcoma High Grade Surface Osteosarcoma
  21. 21.  Origin: Arises fromouter layer ofperiosteum Usually a low gradetumor with fibroblasticstroma andosteoid/woven bone
  22. 22.  Origin: Arises fromtheinner layer of theperiosteum Low to intermediategrade bone withpredominantchondroblasticdifferentiation
  23. 23. Parosteal Osteosarcoma Periosteal Osteosarcoma
  24. 24.  High Grade Surfaceosteosarcoma
  25. 25.  Very rare High grade confined to the cortex Sites: Diaphysis offemur or tibia
  26. 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. 27.  Malignant transformation of benign conditionPaget diseasefibrous dysplasiabone infarct Arising in dedifferentiated chondrosarcoma
  28. 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. 29.  Etiology not known Associated risk factors –rapid bone growth in youngpre-existing bone lesion in old age Most common - Conventional Osteosarcoma
  30. 30. Thank you

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