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 }}<r/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.

Revision as of 05:46, 19 October 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]<ref>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.<r/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.
    • Distinguishing between these two is important

Images

www:

See also

References