Difference between revisions of "Fibrous dysplasia"

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*Low grade [[fibrosarcoma]].
*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>
*[[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> - Prominent calcified spherules, no associated GNAS mutation
*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
** 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.
** Fibrous dysplasia may be self limited and can be followed with observation or if symptomatic bisphosphonate therapy is an option.
**Distinguishing between these two is important
**S0, distinguishing between these two entities is important


===Images===
===Images===
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Image:Fibrous_dysplasia_-_intermed_mag.jpg | Fibrous dysplasia - intermed. 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:Fibrous_dysplasia_-_high_mag.jpg | Fibrous dysplasia - high mag. (WC/Nephron)
Image:Bone FibrousDysplasia LP CTR.jpg|thumb|Medium power shot with the classic 'Chinese character' bone spicules.
Image:Bone FibrousDysplasia LP CTR.jpg|Medium power shot with the classic 'Chinese character' bone spicules.
Image:Bone FibrousDysplasia Jaw MP3 PA.JPG|thumb|Immature bony trabeculae in a fibrous stroma.
Image:Bone FibrousDysplasia Jaw MP3 PA.JPG|Immature bony trabeculae in a fibrous stroma.
</gallery>
</gallery>
www:
www:
*[http://www.pathologypics.com/pictview.aspx?id=104 Fibrous dysplasia of bone - high mag. (pathologypics.com)].
*[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)].
*[http://www.pathologypics.com/BigPicViewer.aspx?PictureID=103 Fibrous dysplasia of bone - low mag. (pathologypics.com)].
*Modern Pathology[http://www.nature.com/modpathol/journal/v20/n3/images/3800753f1.jpg]
*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==
==See also==

Latest revision as of 01:34, 30 November 2014

Fibrous dysplasia
Diagnosis in short

Fibrous dysplasia. H&E stain.
Synonyms osteitis fibrosa
LM woven bone with odd irregular shapes (often described as "chinese characters"), fibrous tissue around bone, no osteoblastic rimming
LM DDx desmoplastic fibroma, low-grade fibrosarcoma, low-grade central osteosarcoma
Site bone
Syndromes McCune-Albright syndrome
Prevalence uncommon
Prognosis benign

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.

Microscopic

Features:[1]

  • Woven bone with odd irregular shapes - key feature.
    • Described as "chinese characters".[2]
  • Fibrous tissue around bone.

Notes:

  • No osteoblastic rimming.

DDx:

  • Desmoplastic fibroma - has lamellar bone.
  • Low grade fibrosarcoma.
  • Low-grade central osteosarcoma.[3]
  • Gnathic ossifying fibroma (Cemento-ossifying fibroma)[4][5] - 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

  • Fibrous dysplasia - low mag. (WC/Nephron)

  • Fibrous dysplasia - intermed. mag. (WC/Nephron)

  • Fibrous dysplasia - high mag. (WC/Nephron)

  • Medium power shot with the classic 'Chinese character' bone spicules.

  • Immature bony trabeculae in a fibrous stroma.

www:

Molecular

  • Postzygotic (somatic) activating mutations of the GNAS1 gene 10, which encodes the α-subunit of the stimulatory G-protein Gs[6]
  • Both mono and polyostatic cases.
  • Causative molecular lesion of McCune-Albright and Mazzabraud syndrome[7]

See also

References

  1. ↑ URL: http://www.pathologypics.com/pictview.aspx?id=104. Accessed on: 14 April 2011.
  2. ↑ URL: http://www.pathcases.com/bone_tumors_and_tumor.htm. Accessed on: 31 May 2011.
  3. ↑ Inwards, CY (2001). "Low-grade central osteosarcoma versus fibrous dysplasia". Pathology Case Reviews 6 (1): 22-27. http://journals.lww.com/pathologycasereviews/Fulltext/2001/01000/Low_Grade_Central_Osteosarcoma_Versus_Fibrous.5.aspx.
  4. ↑ http://www.nature.com/modpathol/journal/v20/n3/full/3800753a.html
  5. ↑ Patel, MM.; Wilkey, JF.; Abdelsayed, R.; D'Silva, NJ.; Malchoff, C.; Mallya, SM. (May 2010). "Analysis of GNAS mutations in cemento-ossifying fibromas and cemento-osseous dysplasias of the jaws.". Oral Surg Oral Med Oral Pathol Oral Radiol Endod 109 (5): 739-43. doi:10.1016/j.tripleo.2009.12.016. PMID 20346714.
  6. ↑ Weinstein, LS.; Shenker, A.; Gejman, PV.; Merino, MJ.; Friedman, E.; Spiegel, AM. (Dec 1991). "Activating mutations of the stimulatory G protein in the McCune-Albright syndrome.". N Engl J Med 325 (24): 1688-95. doi:10.1056/NEJM199112123252403. PMID 1944469.
  7. ↑ http://ghr.nlm.nih.gov/condition/mccune-albright-syndrome
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