Difference between revisions of "Osteofibrous dysplasia"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{Osteofibrous dysplasia}}
| Name      = {{PAGENAME}}
| Image      =  
| Image      =  
| Width      =
| Width      =
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| Micro      =
| Micro      =
| Subtypes  =
| Subtypes  =
| LMDDx      =
| LMDDx      = [[adamantinoma]], OFD-like [[adamantinoma]], [[fibrous dysplasia]]
| Stains    =
| Stains    =
| IHC        =
| IHC        =
| EM        =
| EM        =
| Molecular  =Clonal chromosomal abnormalities
| Molecular  = clonal chromosomal abnormalities
| IF        =
| IF        =
| Gross      =
| Gross      =
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| Syndromes  =
| Syndromes  =
| Clinicalhx =
| Clinicalhx =
| Signs      =Tibial swelling, bowing
| Signs      = tibial swelling, bowing
| Symptoms  =
| Symptoms  =
| Prevalence =Rare
| Prevalence = rare
| Bloodwork  =
| Bloodwork  =
| Rads      =Lytic bone lesion
| Rads      = lytic bone lesion
| Endoscopy  =
| Endoscopy  =
| Prognosis  =Self limited
| Prognosis  = self limited
| Other      =May be related to adamantinoma
| Other      = may be related to adamantinoma
| ClinDDx    =Adamantinoma, Fibrous dysplasia, Metaphyseal fibrous defect, Osteosarcoma
| ClinDDx    = [[adamantinoma]], [[fibrous dysplasia]], [[metaphyseal fibrous defect]], [[osteosarcoma]]
| Tx        =Observation
| Tx        = observation
}}
}}
'''Osteofibrous dysplasia''' is a rare benign condition that afflicits the long bones.<ref name=pmid1563159>{{Cite journal  | last1 = Wang | first1 = JW. | last2 = Shih | first2 = CH. | last3 = Chen | first3 = WJ. | title = Osteofibrous dysplasia (ossifying fibroma of long bones). A report of four cases and review of the literature. | journal = Clin Orthop Relat Res | volume =  | issue = 278 | pages = 235-43 | month = May | year = 1992 | doi =  | PMID = 1563159 }}</ref>


==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>
It is also known as '''ossifying fibroma of long bones'''<ref name=pmid1563159/> and '''congenital osteitis fibrosa'''.
*Rare, benign fibro-osseous lesion


*Alternate names
==General==
**Congenital osteitis fibrosa
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>
*Benign fibro-osseous lesion.
*Rare.<ref name=pmid1563159/>


*Location
Alternate names.
**Cortex of the tibial diaphysis
*Congenital osteitis fibrosa.


*Pathophysiology
Location:
**May be either a clonal neoplastic lesion or a developmental dysplasia.
*Cortex of the tibial diaphysis
**May be related to adamantinoma


*Presentation
Pathophysiology:
**Lower leg swelling
*May be either a clonal neoplastic lesion or a developmental dysplasia.
**Pain
*May be related to adamantinoma
**Tibial bowing


*Radiology
Presentation:
**Intracortical lytic lesion
*Lower leg swelling.
**Surrounding zone of sclerosis
*Pain.
**Seldom progresses radiologically
*Tibial bowing


*Clinical DDX
Radiology:
**Adamantinoma - usually older (teens-twenties), may have soft tissue extension
*Intracortical lytic lesion.
**Metaphyseal fibrous defect - metaphyseal location
*Surrounding zone of sclerosis.
**Fibrous dysplasia
*Seldom progresses radiologically.


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


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


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


==Images==
===Images===
*OFD-Tumor Library| [http://www.tumorlibrary.com/case/images/1326.jpg]
*OFD-Tumor Library| [http://www.tumorlibrary.com/case/images/1326.jpg]
*OFD-Pathology Outlines|  [http://pathologyoutlines.com/wick/osteofibrous%20dysplasia%20(Campanacci%20disease)%20micro002.jpg]
*OFD-Pathology Outlines|  [http://pathologyoutlines.com/wick/osteofibrous%20dysplasia%20(Campanacci%20disease)%20micro002.jpg]
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==Stains==
==Stains==
Not relevant
*Not relevant
 
==IHC==
==IHC==
Keratin positive isolated cells accepted by some.
*Keratin positive - isolated cells accepted by some.
 
==Molecular==
==Molecular==
Clonal chromosomal abnormalities
Clonal chromosomal abnormalities
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==Sign out==
==Sign out==
BONE; CURRETTAGE:  OSTEOFIBROUS DYSPLASIA.
<pre>
BONE, CURRETTAGE:   
- OSTEOFIBROUS DYSPLASIA.
</pre>
==See also==
==See also==
*Tumor Library |[http://www.tumorlibrary.com/case/list.jsp?case_id=95]
*Tumor Library |[http://www.tumorlibrary.com/case/list.jsp?case_id=95]

Latest revision as of 00:17, 2 February 2015

Osteofibrous dysplasia
Diagnosis in short
LM DDx adamantinoma, OFD-like adamantinoma, fibrous dysplasia
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

Osteofibrous dysplasia is a rare benign condition that afflicits the long bones.[1]

It is also known as ossifying fibroma of long bones[1] and congenital osteitis fibrosa.

General

Features:[2]

  • Benign fibro-osseous lesion.
  • Rare.[1]

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.

Notes:

  • The diagnosis depends on the ratio of fibrous to epithelial tissue which can vary within each particular tumour.
    • Due to the above ample tissue is required for accurate histologic diagnosis.
  • Current discussion regarding the definition of an OFD-like adamantinoma.
    • As of 2010, WHO requires 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.

DDx:

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. ↑ 1.0 1.1 1.2 Wang, JW.; Shih, CH.; Chen, WJ. (May 1992). "Osteofibrous dysplasia (ossifying fibroma of long bones). A report of four cases and review of the literature.". Clin Orthop Relat Res (278): 235-43. PMID 1563159.
  2. ↑ "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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