Difference between revisions of "Osteofibrous dysplasia"
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==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 | |||
==References== | ==References== | ||
{{Reflist|1}} | {{Reflist|1}} | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
Revision as of 04:10, 19 October 2014
| Template:Osteofibrous dysplasia | |
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| Diagnosis in short |
==General== [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
- Adamantinoma - usually older (teens-twenties), may have soft tissue extension
- Metaphyseal fibrous defect - metaphyseal location
- Fibrous dysplasia
- 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.
- Current discussion regarding the definition of an OFD-like 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 | http://www.tumorlibrary.com/case/images/1326.jpg
- 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
%20micro002.jpg){kind=link}
%20keratin%20immunost.jpg){kind=link}
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 |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