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Introduction
  • Rare form of fibrous dysplasia that primary affects the tibia and is confined to the cortices
    • also known as ossifying fibroma and Campanacci lesion
  • Epidemiology
    • demographics
      • usually found in younger children (< 10 years old)
      • males>females
    • location
      • primarily in anterior tibia
  • Genetics
    • trisomy 7, 8, 12, 22 have been reported
    • does NOT have Gs alpha activating mutation like fibrous dysplasia has
  • Prognosis
    • lesions usually regress and do not cause problems in adulthood
Presentation 
  • Symptoms 
    • may be asymptomatic 
    • painless swelling
    • anterior or anterolateral bowing of the tibia 
    • pseudoarthrosis develops in 10-30% of patients
  • Physical exam
    • may have local tenderness over the tibia
Imaging
  • Radiographs
    • recommended views
      • AP and lateral of affected area
    • findings
      • anterior eccentric lytic tibial lesion in child that often leads to tibial bowing   
      • usually diaphyseal
      • no periosteal reaction
      • confined to anterior cortex
    • radiographic differential includes adamantinoma
Studies
  • Histology  
    • histology similar to fibrous dysplasia EXCEPT osteoblastic rimming is present  
      • fibroblast proliferation surrounding islands of woven bone with osteoblastic rimming
      • mitotic figures are common
      • may have giant cells
Treatment
  • Nonoperative
    • observation 
      • indication
        • first line of treatment
        • alone is the treatment for most patients
    • bracing
      • indication
        • if deformity significant and interfering with walking
  • Operative
    • deformity correction with osteotomy
      • indication
        • rarely needed
        • significant deformity
        • perform after skeletal maturity 
Differentials & Groups
 
Tibial diaphysis lesion
     
Treatment is Observation alone (2)
   
Osteofibrous dysplasia
     
   
Fibrous dysplasia        
   
Adamantinoma
           
Eosinophilic granuloma        
   
Enchondroma / Olliers / Marfucci's        
   
Osteochondroma / MHE        
   
NOF / Jaffe-Campanacci syndrome        
   
Paget's        
   
ASSUMPTIONS: (1) Younger patient is > 40 yrs; (2) assuming aymptomatic and no impending fracture
 
IBank
 
Location
Age
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)
Case A tibia
12 yrs.
 
   
(1) histology does not always correspond to clinical case 

 

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