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Bone: Chondromyxoid fibroma

Written2003-07Salvatore Romeo, Pancras CW Hogendoorn
Leiden University Medical Center, P.O. box 9600, L1-Q, 2300 RC Leiden, The Netherlands

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Identity

ICD-Topo C400-C403,C408-C414,C418-C419 BONES & JOINTS
ICD-Morpho 9241/0 Chondromyxoid fibroma
Atlas_Id 5149
Phylum Bones::Chondromyxoid fibroma
Note Chondromyxoid fibroma is a benign cartilaginous bone tumour composed of lobules of chondroid and myxoid matrix formed by spindle or stellate shaped cells.
Radiology of Chondromyxoid fibroma.
Fig. 1: Plain film radiograph shows lytic, oval, bubbly lesion with sclerotic rims.

Clinics and Pathology

Epidemiology Chondromyxoid fibroma is a very rare neoplasm accounting for less than 1% of all bone tumours, and less of 2% of all benign bone tumours. Despite 75% of the cases occur in patients younger than 30 years of age, a wide range is reported (4-79 year) Males are slightly more commonly affected.
Clinics A long story of mild pain is reported at the first clinical examination. Bones of the lower extremity are more often affected, with a peculiar predilection for the metaphyseal region. Radiology shows an eccentric lytic lesion (wide size range: 1-14 cm), with a sclerotic rim, and ovoid shape (paralleling the long axis of the bone) with bubbly appearance.
Pathology Histologically the tumours shows a multilobular pattern. The size of the lobules varies from tumour to tumour as well as from field to field. They are composed of fibrous, myxomatous and chondroid areas. However frank hyaline cartilage is less often recognized. The neoplastic cells vary in shape from spindle to stellate, focally atypical nuclei are recognized in 20-30% of the cases. Usually the centre of the lobules is less cellular than the periphery. At the edge of the latter, giant cells can often be recognised. Chunky calcification can be present, especially in older patients.
 
Histological features of Chondromyxoid fibroma.
Fig. 2: Lobules of myxoid matrix are evident at low magnification (Alcian blue stain).
Fig. 3: At higher magnification the neoplastic cells show spindle to stellate shape (Haematoxylin-Eosin stain)
Treatment Simple curettage is the standard treatment.
Evolution The rate of recurrence is 15%.
Prognosis The prognosis is excellent.

Cytogenetics

Cytogenetics
Morphological
Karyotypes of 12 cases are reported in the literature. Complex cytogenic abnormalities are reported. In ten cases chromosome 6 appears to be involved and non-random clonal abnormalities involving 6p25, 6q13 and 6q23 are reported. Of interest 4 cases with peri-centromeric inversion are described [3 cases inv(6)(p13q25) and 1 case inv(6)(p13q23)].

Bibliography

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A recurrent chondromyxoid fibroma with chromosome aberrations ins(5;2)(q13;p21p25) and 2p deletion: a case report.
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Citation

This paper should be referenced as such :
Romeo, S ; Hogendoorn, PCW
Bone: Chondromyxoid fibroma
Atlas Genet Cytogenet Oncol Haematol. 2003;7(3):208-209.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Tumors/ChondroMyxoidFibID5149.html


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

Genes BCLAF1

Translocations implicated (Data extracted from papers in the Atlas)

 inv(6)(p13q25)

External links

Mitelman database inv(6)(p13q25) [Case List]    inv(6)(p13q25) [Association List] Mitelman database (CGAP - NCBI)
arrayMap Topo ( C40,C41) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaseBone: Chondromyxoid fibroma
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed


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