Fibrous dysplasia

Fibrous dysplasia
Updated: 07/31/2018
© Jun Wang, MD, PhD

General features
  • Developmental, non-neoplastic disorder of bone-forming mesenchyme
  • Usually begins prior to puberty and grows slowly
  • Slightly more common in men
  • Malignant transformation: Rare, may represent dedifferentiation of low-grade osteosarcoma; may occur after radiation therapy
Clinical presentations
  • Deformities
  • May have pain or swelling
Pathogenesis
  • Bone maturation arrest at the woven bone stage
Most common sites
  • Medulla of diaphysis or metaphysis of craniofacial bones, femur, ribs
Key radiologic findings
  • Lytic lesion in the metaphysis or diaphysis 
  • "Ground glass" appearance
Key morphological features
  • Irregular trabeculae of metaplastic woven bone (never matures)
  • Hypocellular, fibroblastic stroma
Genetic abnormality
  • Probably activating point mutation (possibly a submit of GNAS1), resulting elevated intracellular cyclic adenosine monophosphates (cAMP)
Treatment
  • Follow up
  • Curettage, bone grafting, and stabilization for patients with symptoms (pain, deformity) or fracture


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