Difference between revisions of "Osteofibrous dysplasia"

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| Micro      =
| Micro      =
| Subtypes  =
| Subtypes  =
| LMDDx      =
| LMDDx      = [[adamantinoma]], OFD-like [[adamantinoma]], [[fibrous dysplasia]]
| Stains    =
| Stains    =
| IHC        =
| IHC        =
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'''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>
'''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>


It is also known as '''ossifying fibroma of long bones'''<ref name=pmid1563159> and '''congenital osteitis fibrosa'''.
It is also known as '''ossifying fibroma of long bones'''<ref name=pmid1563159/> and '''congenital osteitis fibrosa'''.


==General==  
==General==  
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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.
**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.
**Therefore, ample tissue is required for accurate histologic diagnosis


===Images===
===Images===

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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