Updated: 10/5/2016

Non-ossifying Fibroma

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https://upload.orthobullets.com/topic/8027/images/Case B - femur - xray - Parsons_moved.png
https://upload.orthobullets.com/topic/8027/images/Case B - femur - bone scan - Parsons_moved.png
https://upload.orthobullets.com/topic/8027/images/Histology A - low power - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8027/images/5a_moved.jpg
https://upload.orthobullets.com/topic/8027/images/Histology B - high power - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8027/images/Histology C - low power - Parsons_moved.jpg
Introduction
  • Non-ossifying Fibroma (NOF) is a benign fibrogenic lesion that is the most common benign bone tumor in childhood
    • related to dysfunctional ossification
    • other names
      • metaphyseal fibrous defect
      • nonosteogenic fibroma
      • cortical desmoid
      • fibrous cortical defect
      • fibromatosis
      • fibroxanthoma
  • Epidemiology
    • demographics
      • common in children 5-15 years old 
      • 30% of children with open physis have a NOF
    • locations
      • metaphysis of long bones 
      • 80% in lower extremity
        • distal femur > proximal tibia > proximal fibula > distal tibia
        • uncommon in proximal femur, proximal humerus
  • Pathophysiology
    • possibly due to abnormal osteoclastic resorption at the subperiosteal level during remodeling of the metaphysis
  • Associated conditions
    • Jaffe-Campanacci syndrome
      • congenital syndrome of multiple non-ossifying fibromas and
        • cafe au lait pigmentation
        • mental retardation
        • heart, eyes, gonads involved
    • neurofibromatosis
    • familial multifocal NOF
    • ABC
Presentation
  • Symptoms
    • asymptomatic and found incidentally
    • painless
    • may present with pathologic fracture
Imaging
  • Radiographs
    • diagnostic
    • metaphyseal eccentric "bubbly" lytic lesion surrounded by sclerotic rim  
    • cortex may be expanded and thin
    • as bone grows
      • migrates to diaphysis
      • lesions enlarge (1-7cm)
    • lesions become sclerotic as patient approaches skeletal maturity
    • avulsion of adductor magnus insertion in the posteromedial aspect of the distal femur may produce a similar looking lesion. 
  • CT
    • quantitative CT shown to be useful in predicting fracture risk
Studies
  • Histology
    • classic characteristics are  
      • fibroblastic spindle cells in whirled or storiform pattern (helicopter in wheat field) 
      • fibroblastic connective tissue background
      • numerous lipophages and giant cells 
      • hemosiderin pigmentation 
      • occasional ABC component
Differential
  • Giant cell tumor
    • painful
    • rare in skeletally immature
    • no mineralization
  • Osteosarcoma
    • painful
    • irregular zone of bony destruction with less defined zone of transition
    • periosteal reaction
    • mineralized soft tissue mass
Treatment
  • Nonoperative
    • observation     
      • indications
        • first line of treatment
        • most lesions resolve spontaneously and progressively reossify as child enters 2nd and 3rd decade of life
      • technique
        • radiographs at 6, 12months, then annually until reossified
    • casting
      • indication
        • pathologic fracture
        • can be treated as per the fracture alone (long leg casting for distal femur pathologic fx)
  • Operative
    • curettage and bone grafting
      • indication
        • symptomatic and large lesion
        • increased risk of fracture shown on quantitative CT
Differentials & Groups
 
"Bubbly" lytic lesion on xray
 
Hemosiderin seen on Histology
 
Treatment is Observation alone (1)
NOF
 
 
ABC
       
UBC
       
PVNS    
   
Fibrous dysplasia        
Enchondroma        
Osteochondroma        
Eosinophillic granuloma        
Paget's        
ASSUMPTIONS: (1) assuming aymptomatic and no impending fracture
 
IBank
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)
Case A multiple
 
 
 
Case B femur
 
Case C tibia
 
 
 
Case D tibia
 
 
 
 
Case E femur
 
 
 
 
Case F tibia with fx
 
 
 
Case G tibia
 
 
 
 
Case H tibia
 
 
 
 
(1) - histology does not always correspond to case 


 

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Questions (17)
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(OBQ05.252) A 14-year-old child is referred to your office for evaluation of a tibia lesion found incidentally after a minor ankle injury. A radiograph of the child's ankle is shown in Figure A. What treatment do you suggest? Review Topic

QID: 1138
FIGURES:
1

Endocrine consultation secondary to associated endocrine abnormalities

2%

(6/397)

2

Surgical consultation secondary to associated gastrointestional cancers

1%

(3/397)

3

Short leg cast and non-weight bearing for a minimum of 6 weeks

2%

(7/397)

4

Open biopsy and tumor staging

8%

(32/397)

5

Routine followup of tibial lesion

87%

(344/397)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

(OBQ08.143) You are asked to see an 16-year-old patient by his pediatrician after a lesion is found in the child's distal fibula by radiographs taken for a sprained ankle (Figure A). The child is otherwise healthy, active, and has no pain or limitation of motion. Your management should consist of: Review Topic

QID: 529
FIGURES:
1

Non-weight bearing short leg cast

1%

(18/1415)

2

Tumor staging including chest CT, bone scan, MRI of entire bone

1%

(17/1415)

3

Contacting local child protective services

0%

(3/1415)

4

Activities as tolerated, repeat radiographs in 3 to 6 months

94%

(1331/1415)

5

Curettage and allograft bone packing to lesion.

3%

(37/1415)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(OBQ07.43) A 6-year-old boy falls off the monkey bar and presents to the emergency room with an abrasion on his knee and mild knee pain. He is able to bear weight without discomfort and has full range of knee motion. A plain radiograph is shown in Figure A. What is the most appropriate next step in management? Review Topic

QID: 704
FIGURES:
1

CBC, ESR, CRP with bone aspiration for gram stain and culture

2%

(36/1840)

2

Biopsy with neoadjuvant chemotherapy followed by limb salvage surgical resection and adjuvant chemotherapy

2%

(36/1840)

3

Repeat radiographs in 3 months

90%

(1655/1840)

4

Biopsy with external beam irradiation followed by limb salvage surgical resection

0%

(8/1840)

5

MRI and CT scan of the chest

5%

(95/1840)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

(OBQ12.273) An 18-year-old male presents with leg pain after tripping during a soccer game. He has no history of leg pain or trauma. Which of the following is the most likely diagnosis and recommended treatment for the finding seen in his radiograph in figure A? Review Topic

QID: 4633
FIGURES:
1

Enchondroma, observation

4%

(181/4142)

2

Enchondroma, surgical biopsy

1%

(45/4142)

3

Nonossifying fibroma, observation

90%

(3727/4142)

4

Nonossifying fibroma, surgical biopsy

1%

(46/4142)

5

Aneurysmal bone cyst, curettage and bone grafting

3%

(128/4142)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

(OBQ06.96) A 20-year-old man falls while skiing and complains of knee pain. When he presents to the office 2 weeks later his physical exam is normal and his pain has resolved. Radiographs are shown in Figures A & B. What is the next most appropriate step in management? Review Topic

QID: 207
FIGURES:
1

Reassurance and weightbearing as tolerated

87%

(955/1102)

2

Needle biopsy

6%

(66/1102)

3

Incisional biopsy

6%

(62/1102)

4

Excisional biopsy

1%

(11/1102)

5

Wide resection and reconstruction

0%

(3/1102)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 1

(OBQ10.112) A 9-year-old boy injures his ankle while jumping on a trampoline and cannot bear weight on the extremity. A radiograph taken in the emergency room is displayed in Figure A. A biopsy of this lesion would most likely be consistent with which of the following histology slides? Review Topic

QID: 3206
FIGURES:
1

Figure B

3%

(48/1749)

2

Figure C

7%

(119/1749)

3

Figure D

5%

(87/1749)

4

Figure E

34%

(588/1749)

5

Figure F

51%

(894/1749)

ML 4

Select Answer to see Preferred Response

PREFERRED RESPONSE 5
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