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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Fibrous_dysplasia_-_intermed_mag.jpg | |||
| Width = | |||
| Caption = Fibrous dysplasia. [[H&E stain]]. | |||
| Synonyms = osteitis fibrosa | |||
| Micro = woven bone with odd irregular shapes (often described as "chinese characters"), fibrous tissue around bone, no osteoblastic rimming | |||
| Subtypes = | |||
| LMDDx = [[desmoplastic fibroma]], low-grade [[fibrosarcoma]], [[low-grade central osteosarcoma]] | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[bone]] | |||
| Assdx = | |||
| Syndromes = [[McCune-Albright syndrome]] | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = benign | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''Fibrous dysplasia''', also '''osteitis fibrosa''', is a rare disorder of [[bone]] that has a distinctive microscopic appearance. | |||
==General== | |||
Classification: | |||
*''Monostotic'' - one bone involved, ~80% of cases. | |||
*''Polyostotic'' - several bones involved, ~20% of cases. | |||
**May be associated with [[McCune-Albright syndrome]]. | |||
==Microscopic== | |||
Features:<ref>URL: [http://www.pathologypics.com/pictview.aspx?id=104 http://www.pathologypics.com/pictview.aspx?id=104]. Accessed on: 14 April 2011.</ref> | |||
*Woven bone with odd irregular shapes - '''key feature'''. | |||
**Described as "chinese characters".<ref>URL: [http://www.pathcases.com/bone_tumors_and_tumor.htm http://www.pathcases.com/bone_tumors_and_tumor.htm]. Accessed on: 31 May 2011.</ref> | |||
*Fibrous tissue around bone. | |||
Notes: | |||
*No osteoblastic rimming. | |||
DDx: | |||
*[[Desmoplastic fibroma]] - has lamellar bone. | |||
*Low grade [[fibrosarcoma]]. | |||
*[[Low-grade central osteosarcoma]].<ref name=inwards>{{cite journal |author=Inwards, CY |title=Low-grade central osteosarcoma versus fibrous dysplasia |journal=Pathology Case Reviews |volume=6 |issue=1 |pages=22-27 |year=2001 |pmid= |doi= |url= http://journals.lww.com/pathologycasereviews/Fulltext/2001/01000/Low_Grade_Central_Osteosarcoma_Versus_Fibrous.5.aspx }}</ref> | |||
*Gnathic ossifying fibroma (Cemento-ossifying fibroma)<ref>http://www.nature.com/modpathol/journal/v20/n3/full/3800753a.html</ref><ref>{{Cite journal | last1 = Patel | first1 = MM. | last2 = Wilkey | first2 = JF. | last3 = Abdelsayed | first3 = R. | last4 = D'Silva | first4 = NJ. | last5 = Malchoff | first5 = C. | last6 = Mallya | first6 = SM. | title = Analysis of GNAS mutations in cemento-ossifying fibromas and cemento-osseous dysplasias of the jaws. | journal = Oral Surg Oral Med Oral Pathol Oral Radiol Endod | volume = 109 | issue = 5 | pages = 739-43 | month = May | year = 2010 | doi = 10.1016/j.tripleo.2009.12.016 | PMID = 20346714 }}</ref> - Prominent calcified spherules, no associated GNAS mutation | |||
** Gnathic ossifying fibroma recurs and needs to be completely enucleated | |||
** Fibrous dysplasia may be self limited and can be followed with observation or if symptomatic bisphosphonate therapy is an option. | |||
**S0, distinguishing between these two entities is important | |||
===Images=== | |||
<gallery> | |||
Image:Fibrous_dysplasia_-_low_mag.jpg | Fibrous dysplasia - low mag. (WC/Nephron) | |||
Image:Fibrous_dysplasia_-_intermed_mag.jpg | Fibrous dysplasia - intermed. mag. (WC/Nephron) | |||
Image:Fibrous_dysplasia_-_high_mag.jpg | Fibrous dysplasia - high mag. (WC/Nephron) | |||
Image:Bone FibrousDysplasia LP CTR.jpg|Medium power shot with the classic 'Chinese character' bone spicules. | |||
Image:Bone FibrousDysplasia Jaw MP3 PA.JPG|Immature bony trabeculae in a fibrous stroma. | |||
</gallery> | |||
www: | |||
*[http://www.pathologypics.com/pictview.aspx?id=104 Fibrous dysplasia of bone - high mag. (pathologypics.com)]. | |||
*[http://www.pathologypics.com/BigPicViewer.aspx?PictureID=103 Fibrous dysplasia of bone - low mag. (pathologypics.com)]. | |||
*Modern Pathology- fibrous dysplasia (left) vs ossifying fibroma (right)[http://www.nature.com/modpathol/journal/v20/n3/images/3800753f1.jpg] | |||
==Molecular== | |||
*Postzygotic (somatic) activating mutations of the GNAS1 gene 10, which encodes the α-subunit of the stimulatory G-protein Gs<ref>{{Cite journal | last1 = Weinstein | first1 = LS. | last2 = Shenker | first2 = A. | last3 = Gejman | first3 = PV. | last4 = Merino | first4 = MJ. | last5 = Friedman | first5 = E. | last6 = Spiegel | first6 = AM. | title = Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. | journal = N Engl J Med | volume = 325 | issue = 24 | pages = 1688-95 | month = Dec | year = 1991 | doi = 10.1056/NEJM199112123252403 | PMID = 1944469 }}</ref> | |||
*Both mono and polyostatic cases. | |||
*Causative molecular lesion of McCune-Albright and Mazzabraud syndrome<ref>http://ghr.nlm.nih.gov/condition/mccune-albright-syndrome</ref> | |||
==See also== | |||
*[[Bone]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Bone]] | |||
[[Category:Diagnosis]] |
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