Osteofibrous dysplasia - Revision history

Michael: /* Microscopic */

2015-02-02T00:17:13Z

Microscopic

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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===

Michael at 00:14, 2 February 2015

2015-02-02T00:14:02Z

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

Michael at 00:09, 2 February 2015

2015-02-02T00:09:14Z

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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==

Michael at 00:09, 2 February 2015

2015-02-02T00:09:04Z

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	\| Tx = observation		\| Tx = observation
	}}		}}
	'''Osteofibrous dysplasia''', also known as '''congenital osteitis fibrosa'''~~, is a rare benign condition that afflicits the bones of the leg (fibula, tibia)~~.		'''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'''.

	==General==		==General==
	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>		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>
	*~~Rare, benign~~ fibro-osseous lesion.		*Benign fibro-osseous lesion.
			*Rare.<ref name=pmid1563159/>

	Alternate names.		Alternate names.

Michael: tweak

2015-02-02T00:03:13Z

tweak

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	\| Tx = observation		\| Tx = observation
	}}		}}
			'''Osteofibrous dysplasia''', also known as '''congenital osteitis fibrosa''', is a rare benign condition that afflicits the bones of the leg (fibula, tibia).

	==General==		==General==

Michael: tweak more

2015-02-02T00:01:17Z

tweak more

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	}}		}}

	==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>		==General==
	*Rare, benign fibro-osseous lesion		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>
			*Rare, benign fibro-osseous lesion.

	*Alternate names		Alternate names.
	**Congenital osteitis fibrosa		*Congenital osteitis fibrosa.

	*Location		Location:
	**Cortex of the tibial diaphysis		*Cortex of the tibial diaphysis

	*Pathophysiology		Pathophysiology:
	**May be either a clonal neoplastic lesion or a developmental dysplasia.		*May be either a clonal neoplastic lesion or a developmental dysplasia.
	**May be related to adamantinoma		*May be related to adamantinoma

	*Presentation		Presentation:
	**Lower leg swelling		*Lower leg swelling.
	**Pain		*Pain.
	**Tibial bowing		*Tibial bowing

	*Radiology		Radiology:
	**Intracortical lytic lesion		*Intracortical lytic lesion.
	**Surrounding zone of sclerosis		*Surrounding zone of sclerosis.
	**Seldom progresses radiologically		*Seldom progresses radiologically.

	*Clinical DDX		Clinical DDX:
	**Adamantinoma - usually older (teens-twenties), may have soft tissue extension		*[[Adamantinoma]] - usually older (teens-twenties), may have soft tissue extension.
	**Metaphyseal fibrous defect - metaphyseal location		*[[Metaphyseal fibrous defect]] - metaphyseal location.
	**Fibrous dysplasia		*[[Fibrous dysplasia]].

	*Prognosis		Prognosis:
	**Self limited		*Self limited

	*Population		Population:
	**Children		*Children.
	**First 2 decades of life		*First 2 decades of life.
	**Median 9.5 years		*Median 9.5 years.

	==Gross==		==Gross==

Michael: tweak

2015-02-01T23:59:19Z

tweak

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	{{ Infobox diagnosis		{{ Infobox diagnosis
	\| Name = {{~~Osteofibrous dysplasia~~}}		\| Name = {{PAGENAME}}
	\| Image =		\| Image =
	\| Width =		\| Width =
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	\| IHC =		\| IHC =
	\| EM =		\| EM =
	\| Molecular =~~Clonal~~ chromosomal abnormalities		\| Molecular = clonal chromosomal abnormalities
	\| IF =		\| IF =
	\| Gross =		\| Gross =
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	\| Syndromes =		\| Syndromes =
	\| Clinicalhx =		\| Clinicalhx =
	\| Signs =~~Tibial~~ swelling, bowing		\| Signs = tibial swelling, bowing
	\| Symptoms =		\| Symptoms =
	\| Prevalence =~~Rare~~		\| Prevalence = rare
	\| Bloodwork =		\| Bloodwork =
	\| Rads =~~Lytic~~ bone lesion		\| Rads = lytic bone lesion
	\| Endoscopy =		\| Endoscopy =
	\| Prognosis =~~Self~~ limited		\| Prognosis = self limited
	\| Other =~~May~~ be related to adamantinoma		\| Other = may be related to adamantinoma
	\| ClinDDx =~~Adamantinoma~~, ~~Fibrous~~ dysplasia, ~~Metaphyseal~~ fibrous defect, ~~Osteosarcoma~~		\| ClinDDx = [[adamantinoma]], [[fibrous dysplasia]], [[metaphyseal fibrous defect]], [[osteosarcoma]]
	\| Tx =~~Observation~~		\| 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; 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]

Sarah: /* See also */

2014-10-19T04:46:46Z

Sarah at 04:46, 19 October 2014

2014-10-19T04:46:15Z

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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
	}}		}}

Sarah: /* Molecular */

2014-10-19T04:16:36Z

Molecular

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	==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.		BONE; CURRETTAGE: OSTEOFIBROUS DYSPLASIA.