Updated: 6/21/2021

Osteofibrous Dysplasia

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  • summary
    • Osteofibrous Dysplasia is a rare benign tumor-like condition that primarily affects the cortices of the tibia.
    • The condition typically presents in patients less than 10 years of age with painless anterior shin swelling. 
    • Diagnosis is made by a combination of radiographs showing an anterior eccentric lytic tibial lesion and biopsy showing fibroblast proliferation surrounding islands of woven bone with osteoblastic rimming.
    • Treatment is usually observation alone as lesions usually regress and do not cause problems in adulthood.
  • Epidemiology
    • Demographics
      • usually found in younger children (< 10 years old)
      • males>females
    • Anatomic location
      • primarily in anterior tibia
  • Etiology
    • Genetics
      • trisomy 7, 8, 12, 22 have been reported
      • does NOT have Gs alpha activating mutation like fibrous dysplasia has
  • 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
  • Differential
    • Adamantinoma 
    • Osteofibrous Dysplasia Differential 
      Tibial diaphysis lesion
      Treatment is Observation alone 
      Osteofibrous dysplasia

      Fibrous dysplasia
      Adamantinoma

      Eosinophilic granuloma
      Enchondroma / Olliers / Maffucci's
      Osteochondroma / MHE
      NOF /Jaffe-Campanacci syndrome
      Paget's
  • 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
  • Prognosis
    • Lesions usually regress and do not cause problems in adulthood

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Flashcards (3)
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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?

QID: 4364
FIGURES:
1

Osteofibrous dysplasia

77%

(4554/5912)

2

Nonossifying fibroma

8%

(455/5912)

3

Osteoblastoma

5%

(289/5912)

4

Chondroblastoma

3%

(184/5912)

5

Ewing's sarcoma

6%

(377/5912)

L 2 B

Select Answer to see Preferred Response

(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?

QID: 4629
FIGURES:
1

Observation

64%

(1885/2946)

2

Bracing

8%

(236/2946)

3

Casting and monthly follow up

2%

(65/2946)

4

Curettage and bone grafting

23%

(668/2946)

5

Osteotomy with plate application

3%

(78/2946)

L 3 B

Select Answer to see Preferred Response

(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?

QID: 3112
FIGURES:
1

Non-ossifying fibroma

14%

(268/1896)

2

Aneurysmal bone cyst

5%

(90/1896)

3

Low-grade chondrosarcoma

2%

(45/1896)

4

Osteofibrous dysplasia

65%

(1227/1896)

5

Adamantinoma

14%

(260/1896)

L 3 C

Select Answer to see Preferred Response

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(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?

QID: 333
FIGURES:
1

Fibrous dysplasia

18%

(328/1788)

2

Unicameral bone cyst

3%

(47/1788)

3

Aneurysmal bone cyst

7%

(124/1788)

4

Osteofibrous dysplasia

65%

(1162/1788)

5

Non-ossifying fibroma

7%

(118/1788)

L 3 D

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Evidence (5)
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