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<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> - Prominent calcified spherules, no associated GNAS mutation
** 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


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

Revision as of 05:40, 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] - 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