Difference between revisions of "Osteofibrous dysplasia"

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==General== <ref>{{Cite web  | last =  | first =  | title = http://www.smbs.buffalo.edu/ortho/tumorarticles/osteofibrousdysplasiaandadamantinoma.pdf | url = http://www.smbs.buffalo.edu/ortho/tumorarticles/osteofibrousdysplasiaandadamantinoma.pdf | publisher =  | date =  | accessdate = 18 October 2014 }}</ref>
==General==  
*Rare, benign fibro-osseous lesion
Features:<ref>{{Cite web  | last =  | first =  | title = http://www.smbs.buffalo.edu/ortho/tumorarticles/osteofibrousdysplasiaandadamantinoma.pdf | url = http://www.smbs.buffalo.edu/ortho/tumorarticles/osteofibrousdysplasiaandadamantinoma.pdf | publisher =  | date =  | accessdate = 18 October 2014 }}</ref>
*Rare, benign fibro-osseous lesion.


*Alternate names
Alternate names.
**Congenital osteitis fibrosa
*Congenital osteitis fibrosa.


*Location
Location:
**Cortex of the tibial diaphysis
*Cortex of the tibial diaphysis


*Pathophysiology
Pathophysiology:
**May be either a clonal neoplastic lesion or a developmental dysplasia.
*May be either a clonal neoplastic lesion or a developmental dysplasia.
**May be related to adamantinoma
*May be related to adamantinoma


*Presentation
Presentation:
**Lower leg swelling
*Lower leg swelling.
**Pain
*Pain.
**Tibial bowing
*Tibial bowing


*Radiology
Radiology:
**Intracortical lytic lesion
*Intracortical lytic lesion.
**Surrounding zone of sclerosis
*Surrounding zone of sclerosis.
**Seldom progresses radiologically
*Seldom progresses radiologically.


*Clinical DDX
Clinical DDX:
**Adamantinoma - usually older (teens-twenties), may have soft tissue extension
*[[Adamantinoma]] - usually older (teens-twenties), may have soft tissue extension.
**Metaphyseal fibrous defect - metaphyseal location
*[[Metaphyseal fibrous defect]] - metaphyseal location.
**Fibrous dysplasia
*[[Fibrous dysplasia]].


*Prognosis
Prognosis:
**Self limited
*Self limited


*Population
Population:
**Children
*Children.
**First 2 decades of life
*First 2 decades of life.
**Median 9.5 years
*Median 9.5 years.


==Gross==  
==Gross==  

Revision as of 00:01, 2 February 2015

Osteofibrous dysplasia
Diagnosis in short
Molecular clonal chromosomal abnormalities
Signs tibial swelling, bowing
Prevalence rare
Radiology lytic bone lesion
Prognosis self limited
Other may be related to adamantinoma
Clin. DDx adamantinoma, fibrous dysplasia, metaphyseal fibrous defect, osteosarcoma
Treatment observation


General

Features:[1]

  • Rare, benign fibro-osseous lesion.

Alternate names.

  • Congenital osteitis fibrosa.

Location:

  • Cortex of the tibial diaphysis

Pathophysiology:

  • May be either a clonal neoplastic lesion or a developmental dysplasia.
  • May be related to adamantinoma

Presentation:

  • Lower leg swelling.
  • Pain.
  • Tibial bowing

Radiology:

  • Intracortical lytic lesion.
  • Surrounding zone of sclerosis.
  • Seldom progresses radiologically.

Clinical DDX:

Prognosis:

  • Self limited

Population:

  • Children.
  • First 2 decades of life.
  • Median 9.5 years.

Gross

Microscopic

The lesion has a zonal architecture with a center of immature bone surrounded by more mature lamellar bone. The central spicules of woven bony trabeculae are lined by a layer of osteoblasts. The backgound is a loose and storiform fibrous tissue.

  • Histologic DDX
    • Adamantinoma - epithelial elements are prominent and atypical
    • OFD-like adamantinoma (see below)
    • Fibrous dysplasia - Bony trabeculae lack osteoblastic rimming, not zonal
  • Tips
    • Current discussion regarding the definition of an OFD-like adamantinoma.
      • WHO as of 2010 required absence of keratin-positive cells for OFD
      • Some accept as OFD lesions with scattered isolated keratin positive spindled cells.
      • Gray zone and varying interpretations of the boundry of OFD, OFD-like adamantinoma and adamantinoma.
    • The diagnosis depends on the ratio of fibrous to epithelial tissue which can vary within each particular tumor.
    • Therefore, ample tissue is required for accurate histologic diagnosis

Images

  • OFD-Tumor Library| [1]
  • OFD-Pathology Outlines| [2]
  • OFD-Pathology Outlines | [3]
  • OFD-Pathology Outlines | [4]
  • OFD-Sarcoma Images | [5]
  • OFD-Sarcoma Images | [6]
  • OFD-Sarcoma Images | [7]
  • OFD-Sarcoma Images | [8]

Stains

  • Not relevant

IHC

  • Keratin positive - isolated cells accepted by some.

Molecular

Clonal chromosomal abnormalities

  • Trisomies of chromosomes 7, 8, 12,21, and/or 22

Sign out

BONE, CURRETTAGE:  
- OSTEOFIBROUS DYSPLASIA.

See also

  • Tumor Library |[9]
  • Sarcoma Images |[10]

References

  1. ↑ "http://www.smbs.buffalo.edu/ortho/tumorarticles/osteofibrousdysplasiaandadamantinoma.pdf". http://www.smbs.buffalo.edu/ortho/tumorarticles/osteofibrousdysplasiaandadamantinoma.pdf. Retrieved 18 October 2014.
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