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Bone: Fibrous dysplasia of the bone

Written2001-06Paola Dal Cin
Department of Pathology, Brigham, Women's Hospital, 75 Francis Street, Boston, MA 02115, USA

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C400-C403,C408-C414,C418-C419 BONES & JOINTS
Atlas_Id 5093
Phylum Bone tumors:Tumors of undefined neoplastic nature:Fibrous dysplasia
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
  • It is characterized by a broad clinical spectrum, varying from the more frequent solitary (monostatic) asymptomatic lesion to extensive and severe multifocal (polyostatic) lesions. The monostotic variant most commonly affects the ribs, femur and tibia of older children and young adults. The less frequent polyostotic type may be associated with endocrine abnormalities, skin hyperpigmentation and soft tissue myxoma (so-called Albright syndrome).
  • On radiographs, fibrous dysplasia presents as a well defined osteolytic lesion with benign features, centered within the medulla, and frequently bordered by a shell of reactive sclerosis. Depending on the relative proportion of osseous to fibrous tissue, the lesion can be completely lytic or, most typically, show a relatively high density, the so-called ïground-glass' appearance in a least part of it. Especially in bones with small diameter and flat bones, fibrous dysplasia frequently expands the bone.
  • 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).

    Bibliography

    Desmoplastic fibroma arising in fibrous dysplasia. Chromosomal analysis and review of the literature.
    Bridge JA, Rosenthal H, Sanger WG, Neff JR
    Clinical orthopaedics and related research. 1989 : 272-278.
    PMID 2676299
     
    Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone.
    Bridge JA, Swarts SJ, Buresh C, Nelson M, Degenhardt JM, Spanier S, Maale G, Meloni A, Lynch JC, Neff JR
    The American journal of pathology. 1999 ; 154 (3) : 729-733.
    PMID 10079250
     
    Fibrous dysplasia and the short arm of chromosome 12.
    Dal Cin P, Bertoni F, Bacchini P, Hagemeijer A, Van den Berghe H
    Histopathology. 1999 ; 34 (3) : 279-280.
    PMID 10217576
     
    Recurrent chromosome aberrations in fibrous dysplasia of the bone: a report of the CHAMP study group. CHromosomes And MorPhology.
    Dal Cin P, Sciot R, Brys P, De Wever I, Dorfman H, Fletcher CD, Jonsson K, Mandahl N, Mertens F, Mitelman F, Rosai J, Rydholm A, Samson I, Tallini G, Van den Berghe H, Vanni R, Willen H
    Cancer genetics and cytogenetics. 2000 ; 122 (1) : 30-32.
    PMID 11104029
     
    Bone Tumors
    Dorfman HD, Czerniak B
    The Mosby Publisher. 1998.
     
    Clonal structural chromosome aberrations in fibrous dysplasia.
    Mertens F, Albert A, Heim S, Lindholm J, Brosjö O, Mitelman F, Mandahl N
    Genes, chromosomes & cancer. 1994 ; 11 (4) : 271-272.
    PMID 7533532
     
    Malignancies in fibrous dysplasia.
    Ruggieri P, Sim FH, Bond JR, Unni KK
    Cancer. 1994 ; 73 (5) : 1411-1424.
    PMID 8111708
     
    Cytogenetic study of 249 consecutive patients examined for a bone tumor.
    Tarkkanen M, Kaipainen A, Karaharju E, Böhling T, Szymanska J, Heliö H, Kivioja A, Elomaa I, Knuutila S
    Cancer genetics and cytogenetics. 1993 ; 68 (1) : 1-21.
    PMID 8330278
     
    Dahlin's Bone Tumors. General Aspects and Data on 11.087 cases
    Unni KK
    5th ed. 1996.
     

    Citation

    This paper should be referenced as such :
    Dal, Cin P
    Bone: Fibrous dysplasia of the bone
    Atlas Genet Cytogenet Oncol Haematol. 2001;5(3):215-216.
    Free journal version : [ pdf ]   [ DOI ]
    On line version : http://AtlasGeneticsOncology.org/Tumors/FibDysplasiaBoneID5093.html


    Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

    Genes GNAS

    External links

    arrayMap Topo ( C40,C41) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
     
     
    Disease databaseBone: Fibrous dysplasia of the bone
    REVIEW articlesautomatic search in PubMed
    Last year articlesautomatic search in PubMed


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