Osteofibrous dysplasia
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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 |
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:
- 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]
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
References
- ↑ 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.
- ↑ "http://www.smbs.buffalo.edu/ortho/tumorarticles/osteofibrousdysplasiaandadamantinoma.pdf". http://www.smbs.buffalo.edu/ortho/tumorarticles/osteofibrousdysplasiaandadamantinoma.pdf. Retrieved 18 October 2014.