Desmoplastic Fibroma of Bone
Synonyms: Desmoid tumour of bone
Intraosseous counterpart of soft tissue desmoid
Quick Facts
Behaviour
Intermediate
Category
Bone
Synonyms
Desmoid tumour of bone
Category
Bone
Behaviour
Intermediate
Gender
M = F
Tissue of Origin
Fibrous
Epidemiology
- Rare intraosseous fibrous tumour (<0.1% of primary bone tumours)
- Young adults and adolescents (peak incidence 10-30 years)
Clinical Features
- Pain and swelling
- May cause pathological fracture
- Slowly growing
Location
- Jaw (mandible)
- Long bones (femur, radius, ulna)
- Pelvis
Imaging
- Plain Radiograph: lytic lesion with trabeculated pattern and cortical thinning
- MRI: low signal on T1 and T2 due to high collagen content
Pathology
- Hypocellular fibrous tissue with abundant collagen
- No atypia or mitoses
- Similar to soft tissue desmoid fibromatosis
Treatment
- Wide surgical excision
- Recurrence common if incomplete excision
Prognosis
- No metastatic potential
- High local recurrence rate (40%) if incomplete resection
Key Points
- Intraosseous counterpart of soft tissue desmoid
- CTNNB1/APC pathway alterations
- High local recurrence - wide excision essential
Workup - Blood Tests
None specific
Workup - Local Imaging
- Plain radiograph
- MRI with contrast of primary site
Workup - Biopsy
Core needle biopsy
Workup - Staging
Not required
Workup - Other
Evaluation of the mandible/maxilla if head and neck involvement is suspected
Follow-up Summary
Consider lonter term follow up as late recurrence is reported
Medical disclaimer
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