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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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Questions (5)

(OBQ12.4) A 9-year-old male presents with a mass on his lower leg. He denies any history of trauma, and is otherwise healthy. A current radiograph of the affected leg is shown in Figure A. A biopsy of the lesion is obtained, and is shown in Figures B and C. What is the most likely diagnosis in this patient? Review Topic

QID: 4364
FIGURES:
1

Osteofibrous dysplasia

76%

(3336/4387)

2

Nonossifying fibroma

8%

(357/4387)

3

Osteoblastoma

5%

(231/4387)

4

Chondroblastoma

3%

(143/4387)

5

Ewing's sarcoma

6%

(284/4387)

Select Answer to see Preferred Response

PREFERRED RESPONSE 1

(OBQ12.269) A 10-year-old male presents for evaluation of a painless deformity of his lower leg. Based on the radiograph and histology slide shown in Figures A and B, what is the next best step for this patient? Review Topic

QID: 4629
FIGURES:
1

Observation

62%

(1286/2091)

2

Bracing

9%

(195/2091)

3

Casting and monthly follow up

2%

(48/2091)

4

Curettage and bone grafting

24%

(496/2091)

5

Osteotomy with plate application

3%

(57/2091)

Select Answer to see Preferred Response

PREFERRED RESPONSE 1

(OBQ10.24) A 12-year-old female complains of a prominence below her left knee. It is tender when bumped, but painless at rest and with everyday activities. She denies fatigue, weight loss, and any other masses. Physical exam is notable for mild tenderness with firm palpation of the bump. She has full painless range of motion of the knee and ankle. A radiograph, CT scan, and histology slide are provided in Figures A, B, and C respectively. Which of the following is the most likely diagnosis? Review Topic

QID: 3112
FIGURES:
1

Non-ossifying fibroma

13%

(173/1325)

2

Aneurysmal bone cyst

3%

(45/1325)

3

Low-grade chondrosarcoma

2%

(32/1325)

4

Osteofibrous dysplasia

66%

(881/1325)

5

Adamantinoma

14%

(191/1325)

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(OBQ06.147) A 3-year-old boy was referred to you for evaluation of a bowing deformity of his leg and a persistent limp. Radiograph is shown. What is the most likely diagnosis? Review Topic

QID: 333
FIGURES:
1

Fibrous dysplasia

19%

(236/1253)

2

Unicameral bone cyst

3%

(34/1253)

3

Aneurysmal bone cyst

6%

(79/1253)

4

Osteofibrous dysplasia

65%

(809/1253)

5

Non-ossifying fibroma

7%

(87/1253)

Select Answer to see Preferred Response

PREFERRED RESPONSE 4
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