Difference between revisions of "Osteofibrous dysplasia"
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{ | | Name = {{PAGENAME}} | ||
| Image = | | Image = | ||
| Width = | | Width = | ||
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| IHC = | | IHC = | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = clonal chromosomal abnormalities | ||
| IF = | | IF = | ||
| Gross = | | Gross = | ||
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| 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 | ||
}} | }} | ||
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**Therefore, ample tissue is required for accurate histologic diagnosis | **Therefore, ample tissue is required for accurate histologic diagnosis | ||
==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 | <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] | ||
Revision as of 23:59, 1 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== [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-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]
![[1]](http://www.tumorlibrary.com/case/images/1326.jpg){kind=link}
![[2]](http://pathologyoutlines.com/wick/osteofibrous%20dysplasia%20(Campanacci%20disease)%20micro002.jpg){kind=link}
![[3]](http://pathologyoutlines.com/wick/osteofibrous%20dysplasia%20(Campanacci%20disease)%20keratin%20immunost.jpg){kind=link}
![[4]](http://pathologyoutlines.com/images/bones/182.jpg){kind=link}
![[5]](http://www.sarcomaimages.com/images/db/osteofibrous-dysplasia/812-sample-case/186-osteofibrous-dysplasia-with-aneurismal-bone-cyst-like-changes-6.jpg){kind=link}
![[6]](http://www.sarcomaimages.com/images/db/osteofibrous-dysplasia/812-sample-case/184-osteofibrous-dysplasia-with-aneurismal-bone-cyst-like-changes-4.jpg){kind=link}
![[7]](http://www.sarcomaimages.com/images/db/osteofibrous-dysplasia/812-sample-case/183-osteofibrous-dysplasia-with-aneurismal-bone-cyst-like-changes-3.jpg){kind=link}
![[8]](http://www.sarcomaimages.com/images/db/osteofibrous-dysplasia/812-sample-case/182-osteofibrous-dysplasia-with-aneurismal-bone-cyst-like-changes-3..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.