DEFINITION AND PATHOGENESIS
- 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
- 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
- 12/17 + for PDGFRA and/or PDGFRB
- (Recurrent coamplification of KIT, PDGFRA, and KDR)
- Losses were most commonly detected at 6q, 8p, 9p, 10, 13q, and 20p
- Gains were seen at 1q, 4q, 5p, 8q, 12p, 15q, 16q, 17q, 20q, 22q (most frequent), and Xp
- PDGF-B gene at 22q12.3-q13.1
- 40% with presence of PDGF-B proteins
- IGF1R at 15q26.3
- CHD1L at 1q21.1
- Ring chromosome
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Fibrous dysplasia
- Fibromatosis (desmoid tumor)
- Mesenchymal chondrosarcoma
- Metastasis, esp. melanoma and carcinoma
DISEASE COURSE AND TREATMENT
- 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%
MULTIPLE DIFFUSE FIBROSARCOMA OF BONE
- Rarely reported presenting with multiple osteolytic lesions
- ? ESR reported (rule out myeloma)
MYOFIBROSARCOMA OF BONE
- Lytic destructive lesions, ± soft tissue extension, distal femur and pelvis reported; age 60-71
- Cellular fascicular/storiform and hypocellular fibrous areas
- + vimentin
- + muscle actin (HHF35)
- + ?-smooth muscle actin
- +calponin and desmin
- high molecular weight caldesmon
- Wide resection required; distant metastases reported
SECONDARY FIBROSARCOMA OF BONE
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