Fibrous dysplasia of bone
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| Fibrous dysplasia | |
|---|---|
| Classification and external resources | |
 Micrograph showing fibrous dysplasia with the characteristic thin, irregular (Chinese character-like) bony trabeculae and fibrotic marrow space. H&E stain. |
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| ICD-10 | K10.8, M85.0, Q78.1 |
| ICD-9 | 756.54 |
| MedlinePlus | 001234 |
| eMedicine | radio/284 |
| MeSH | D005357 |
Fibrous dysplasia is a disease that causes bone thinning[1] and growths or lesions in one or more bones, and leads to bone weakness and scar formation within the bones.[2]
Contents |
[edit] Presentation
These lesions are tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Especially when involving the skull or facial bones, the lesions can cause externally visible deformities. The skull is often, but not necessarily, affected, and any other bone(s) can be involved.
- Many patients have lesions localized in only one bone (monostotic fibrous dysplasia), affecting 70–80%.
- Others have them in many bones (polyostotic fibrous dysplasia).
In 3% of cases, people suffering from fibrous dysplasia also have endocrine diseases and skin pigmentation; the three together constitute McCune-Albright syndrome. These endocrine diseases include precocious (early) puberty, often occurring as early as 6 years old.
[edit] Pathology
In fibrous dysplasia, the marrow space is filled with fibrous tissue. The bony trabeculae are abnormally thin and irregular, and often likened to Chinese characters.
[edit] Causes
Fibrous dysplasia is very rare; not much is known about it, and there is no known cure. Levels of the transcription factor C-fos are raised in fibrous dysplasia, leading to gene over-expression and tumour formation.[3][4] It is not hereditary.[5] There are two types of fibrous dysplasia:
- Monostotic (Involving a single bone, or adjacent bones, like both the upper and lower jaw)
- Polyostotic (Involving many bones).
The most severe form of polyostotic fibrous dysplasia is known as McCune-Albright syndrome. Fibrous dysplasia of bone may also be an associated abnormality in Neurofibromatosis type II.
[edit] See also
[edit] References
- ^ "fibrous dysplasia of bone" at Dorland's Medical Dictionary
- ^ http://www.mayoclinic.com/health/fibrous-dysplasia/DS00991
- ^ Candeliere GA, Glorieux FH, Prud'homme J, St-Arnaud R (June 1995). "Increased expression of the c-fos proto-oncogene in bone from patients with fibrous dysplasia". N. Engl. J. Med. 332 (23): 1546–51. doi:10.1056/NEJM199506083322304. PMID 7739708. http://content.nejm.org/cgi/content/abstract/332/23/1546.
- ^ Kashima TG, Nishiyama T, Shimazu K, et al. (February 2009). "Periostin, a novel marker of intramembranous ossification, is expressed in fibrous dysplasia and in c-Fos-overexpressing bone lesions". Hum. Pathol. 40 (2): 226–37. doi:10.1016/j.humpath.2008.07.008. PMID 18799196. http://linkinghub.elsevier.com/retrieve/pii/S0046-8177(08)00317-1.
- ^ Fibrous Dysplasia at eMedicine
[edit] External links
- Duke Orthopedics fibrous_dysplasia
- Fibrous Dysplasia Pictures MedPix Imaging Database
- Fibrous Dysplasia of the Skull Images radRounds Radiology Image Database
- Fibrous dysplasia from Radiopaedia.org.
- Fibrous Dysplasia Foundation
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