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Fibrosarcoma of Bone


  • Primary fibrosarcoma: a fibroblastic malignancy producing variable amounts of collagen
  • Central: arising from within the intramedullary canal
  • Peripheral: arising from periosteal tissues
  • Secondary fibrosarcoma: arises from a pre-existing lesion (with generally a poorer prognosis)
    • Post XRT
    • Ameloblastic fibroma
    • GCT
    • Fibrous dysplasia
    • Paget's disease
    • Dedifferentiated lwo-grade chondrosarcoma
    • Bone infarct
    • Chronic osteomyelitis
  • Multicentric fibrosarcoma: rule out spindle cell melanoma or carcinoma metastases
  • Congenital fibrosarcoma: extremely rare


  • Early dx and differentiation from benign lesions may be important for an otherwise dismal prognosis
  • <5% of primary malignancies of bone


  • Can present at any age but most likely in the fourth decade of life usually in the lower extremities with the femur and tibia being the most common sites
  • Unusual sites reported:
    • Distal phalanx of a toe in a 12-yr old
  • Can be primary or develop secondary to FD, GCT, XRT, Paget's, bone infarcts, and chronic osteomyelitis
  • Often have a long duration of symptoms prior to their diagnosis
  • Better prognosis if dxd prior to 10 years of age


  • Osteolytic with "motheaten" premeative appearance
  • No sclerosis with cortical destruction
  • Sequestrum may be seen


  • White and firm with marked collagen production
  • Higher grade lesions permeate through cortical bone and are softer and fleshy


  • Spindled fibroblasts with nuclear pleomorphism
  • Not as much collagen present with 'herring bone' pattern
  • Frequent mitoses; sparse giant cells present
  • No osteoid or cartilage production
  • Immunohistochemistry
    • 12/17 + for PDGFRA and/or PDGFRB
      • (Recurrent coamplification of KIT, PDGFRA, and KDR)
  • Molecular


  • NOF
  • Fibrous dysplasia
  • GCT
  • Fibromatosis (desmoid tumor)
  • Osteosarcoma
  • MFH
  • Mesenchymal chondrosarcoma
  • Metastasis, esp. melanoma and carcinoma


  • Tx remains wide excision; chemotx not proven but treat as osteosarcoma
  • Dismal results overall with 1/3 survival rate at 10 years for high grade lesions
  • Prognosis most dependant upon histologic grade
  • 5YS overall reported 28-34% in high grade tumors; 50-83% in low grade tumors
  • Secondary fibrosarcomas carry a more ominous prognosis (10% 10 year survival)
  • Congenital fibrosarcoma has a more favorable prognosis (directly related to age of dx) and may be amenable to intralesional excision + adjuvant (? neoadjuvant better) chemotx (controversial) without recurrence
    • <10 yrs old at dx, metastatic rate @ 5 yrs = 5%
    • < 5 yrs old at dx, metastatic rate @ 5 yrs = 7.3%
  • LR in 43%



    • Rarely reported presenting with multiple osteolytic lesions
    • ? ESR reported (rule out myeloma)

    • Lytic destructive lesions, ± soft tissue extension, distal femur and pelvis reported; age 60-71
    • Cellular fascicular/storiform and hypocellular fibrous areas
    • Immunohistochemistry
      • + vimentin
      • + muscle actin (HHF35)
      • + ?-smooth muscle actin
      • +calponin and desmin
      • high molecular weight caldesmon
    • Wide resection required; distant metastases reported


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