Osteofibrous dysplasia: Difference between revisions

Jump to navigation Jump to search
← Older edit
Sarah (talk | contribs)
653 edits
Michael (talk | contribs)
49,301 edits
 
(12 intermediate revisions by 2 users not shown)
Line 1: Line 1:
{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{Osteofibrous dysplasia}}
| Name      = {{PAGENAME}}
| Image      =  
| Image      =  
| Width      =
| Width      =
Line 7: Line 7:
| Micro      =
| Micro      =
| Subtypes  =
| Subtypes  =
| LMDDx      =
| LMDDx      = [[adamantinoma]], OFD-like [[adamantinoma]], [[fibrous dysplasia]]
| Stains    =
| Stains    =
| IHC        =
| IHC        =
| EM        =
| EM        =
| Molecular  =
| Molecular  = clonal chromosomal abnormalities
| IF        =
| IF        =
| Gross      =
| Gross      =
Line 19: Line 19:
| Syndromes  =
| Syndromes  =
| Clinicalhx =
| Clinicalhx =
| Signs      =
| Signs      = tibial swelling, bowing
| Symptoms  =
| Symptoms  =
| Prevalence =
| Prevalence = rare
| Bloodwork  =
| Bloodwork  =
| Rads      =
| Rads      = lytic bone lesion
| Endoscopy  =
| Endoscopy  =
| Prognosis  =
| Prognosis  = self limited
| Other      =
| Other      = may be related to adamantinoma
| ClinDDx    =
| ClinDDx    = [[adamantinoma]], [[fibrous dysplasia]], [[metaphyseal fibrous defect]], [[osteosarcoma]]
| Tx        =
| 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==  
Line 71: Line 76:
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.
**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.


*Tips
DDx:
**Current discussion regarding the definition of an OFD-like adamantinoma.
*[[Adamantinoma]] - epithelial elements are prominent and atypical.
***WHO as of 2010 required absence of keratin-positive cells for OFD
*OFD-like [[adamantinoma]] - see below.
***Some accept as OFD lesions with scattered isolated keratin positive spindled cells.
*[[Fibrous dysplasia]] - bony trabeculae lack osteoblastic rimming, not zonal.
***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.
===Images===
**Therefore, ample tissue is required for accurate histologic diagnosis
*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)%20keratin%20immunost.jpg]
*OFD-Pathology Outlines | [http://pathologyoutlines.com/images/bones/182.jpg]
*OFD-Sarcoma Images | [http://www.sarcomaimages.com/images/db/osteofibrous-dysplasia/812-sample-case/186-osteofibrous-dysplasia-with-aneurismal-bone-cyst-like-changes-6.jpg]
*OFD-Sarcoma Images | [http://www.sarcomaimages.com/images/db/osteofibrous-dysplasia/812-sample-case/184-osteofibrous-dysplasia-with-aneurismal-bone-cyst-like-changes-4.jpg]
*OFD-Sarcoma Images | [http://www.sarcomaimages.com/images/db/osteofibrous-dysplasia/812-sample-case/183-osteofibrous-dysplasia-with-aneurismal-bone-cyst-like-changes-3.jpg]
*OFD-Sarcoma Images | [http://www.sarcomaimages.com/images/db/osteofibrous-dysplasia/812-sample-case/182-osteofibrous-dysplasia-with-aneurismal-bone-cyst-like-changes-3..jpg]


==Images==
==Stains==
*OFD | http://www.tumorlibrary.com/case/images/1326.jpg
*Not relevant
*OFD | http://pathologyoutlines.com/wick/osteofibrous%20dysplasia%20(Campanacci%20disease)%20micro002.jpg
*OFD | http://pathologyoutlines.com/wick/osteofibrous%20dysplasia%20(Campanacci%20disease)%20keratin%20immunost.jpg
*OFD | http://pathologyoutlines.com/images/bones/182.jpg


==Stains==
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
**Trisomies of chromosomes 7, 8, 12,21, and/or 22
*Trisomies of chromosomes 7, 8, 12,21, and/or 22
 
==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]
*Sarcoma Images |http://www.sarcomaimages.com/sub.php?v=812-sample-case&p=osteofibrous-dysplasia
*Sarcoma Images |[http://www.sarcomaimages.com/sub.php?v=812-sample-case&p=osteofibrous-dysplasia]


==References==
==References==
Retrieved from "https://librepathology.org/wiki/Osteofibrous_dysplasia"