Updated: 6/21/2021

Desmoplastic Fibroma

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Images
https://upload.orthobullets.com/topic/8029/images/Case A - tibia - xray 2 - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8029/images/Case A - tibia - CT - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8029/images/Case A - tibia - xray - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8029/images/Case B - tibia - xray - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8029/images/Case C - femur - xray - Parsons_moved.jpg
  • summary
    • Desmoplastic Fibromas are low-grade malignant fibrogenic tumors of bone that are most commonly found in the metaphysis of long bones. Patients typically present between the ages of 15 and 25 with regional pain and swelling. 
    • Diagnosis is made with a characteristic metaphyseal lytic lesion with soap bubble appearance on radiographs with biopsy showing fibrous spindle cells and mature fibroblasts without cellular atypia.
    • Treatment is usually wide surgical resection to prevent recurrence.
  • Epidemiology
    • Incidence
      • extremely rare
    • Demographics
      • usually occurs in adolescence and young adults (15-25)
    • Anatomic location
      • mandible and metaphysis of long bones
  • Presentation
    • Symptoms
      • painful lesion
  • Imaging
    • Radiographs
      • findings
        • purely lytic lesion
        • soap bubble appearance (internal pseudotrabeculatins)
        • endosteal scalloping
        • may involve cortex
        • may have soft tissue mass
  • Histology
    • Classic characteristics are
      • dense swirling fibrous spindle cells
      • mature fibroblasts without cellular atypia
      • abundant bundles of collagen
  • Treatment
    • Operative
      • wide surgical resection vs. intralesional curettage
        • indications
          • wide surgical resection recommended to avoid recurrence
  • Prognosis
    • Recurrence rate of 40%
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