Bone: Fibrous dysplasia of the bone
2001-06-01 Paola Dal Cin, PhD Affiliation1.Department of Pathology, Brigham, Womens Hospital, 75 Francis Street, Boston, MA 02115, USA
Summary
Note
In the major text-books on bone pathology, fibrous dysplasia (FD) is regarded as a non-neoplastic process. It is included in the chapter on conditions that simulate a bone neoplasm and it is defined elsewhere as a dysplastic disorder of bone.
Clinics and Pathology
Etiology
Unknown
Clinics
Pathology
Microscopically, irregular trabeculae of woven bone are embedded in a moderately cellular fibrous matrix. The bony trabeculae have variable and irregular shapes. Osteoblastic rimming of the trabeculae is characteristically inconspicuous and some of the trabeculae seem to emerge from the surrounding fibrous background, suggesting a metaplastic process. Benign lesions that histologically can be confused with FD are osteofibrous dysplasia of long bones and desmoplastic fibroma. Cases of FD with extensive cartilaginous differentiation may be mistaken histologically or radiologically for a benign chondroblastic tumor, although the adjacent fibro-osseous component allows this distinction. The most important differential diagnosis of FD is with low grade (well-differentiated) central osteosarcoma. Rarely sarcomas can arise in FD and some of them occur in patients that had received radiation therapy (post-radiation sarcomas) .
Treatment
It should be conservative
Cytogenetics
Cytogenetics morphological
Clonal chromosome aberrations have been reported in 8 out of eleven cases of FD in which chromosomal analysis has been performed, suggesting that this entity is of a neoplastic nature. The only recurrent changes described so far in FD have been structural 12p13 aberrations (3 cases) and trisomy 2 (3 cases).
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 2676299 | 1989 | Desmoplastic fibroma arising in fibrous dysplasia. Chromosomal analysis and review of the literature. | Bridge JA et al |
| 10079250 | 1999 | Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone. | Bridge JA et al |
| 10217576 | 1999 | Fibrous dysplasia and the short arm of chromosome 12. | Dal Cin P et al |
| 11104029 | 2000 | Recurrent chromosome aberrations in fibrous dysplasia of the bone: a report of the CHAMP study group. CHromosomes And MorPhology. | Dal Cin P et al |
| 7533532 | 1994 | Clonal structural chromosome aberrations in fibrous dysplasia. | Mertens F et al |
| 8111708 | 1994 | Malignancies in fibrous dysplasia. | Ruggieri P et al |
| 8330278 | 1993 | Cytogenetic study of 249 consecutive patients examined for a bone tumor. | Tarkkanen M et al |
Citation
Paola Dal Cin, PhD
Bone: Fibrous dysplasia of the bone
Atlas Genet Cytogenet Oncol Haematol. 2001-06-01
Online version: http://atlasgeneticsoncology.org/solid-tumor/5093/bone-fibrous-dysplasia-of-the-bone
