Bone: Chondromyxoid fibroma

2003-07-01   Salvatore Romeo , Pancras CW Hogendoorn 

1.Leiden University Medical Center, P.O. box 9600, L1-Q, 2300 RC Leiden, The Netherlands

Summary

Note

Chondromyxoid fibroma is a benign cartilaginous bone tumour composed of lobules of chondroid and myxoid matrix formed by spindle or stellate shaped cells.
Atlas Image
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.
Atlas Image
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)(p25q13) and 1 case inv(6)(p23q13)].

Bibliography

Pubmed IDLast YearTitleAuthors
92294171997Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. I. The intramedullary cartilage tumors.Brien EW et al
126061372003Cytogenetic findings in benign cartilaginous neoplasms.Buddingh EP et al
4980161979Cytophotometric studies of the nuclear DNA content in cartilaginous tumors.Cuvelier CA et al
63371621983Chondromyxoid fibroma. The experience at the Istituto Ortopedico Rizzoli.Gherlinzoni F et al
96485591998Cytogenetic analysis of a scapular chondromyxoid fibroma.Halbert AR et al
127421532003Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: chondrosarcoma and other cartilaginous neoplasms.Sandberg AA et al
94522641998Evidence of an association between 6q13-21 chromosome aberrations and locally aggressive behavior in patients with cartilage tumors.Sawyer JR et al
86497391996Benign cartilage tumors.Scarborough MT et al
117933712002Correlation between clinicopathological features and karyotype in 100 cartilaginous and chordoid tumours. A report from the Chromosomes and Morphology (CHAMP) Collaborative Study Group.Tallini G et al
84536001993A recurrent chondromyxoid fibroma with chromosome aberrations ins(5;2)(q13;p21p25) and 2p deletion: a case report.Tarkkanen M et al
27931601989Chondromyxoid fibroma of bone: thirty-six cases with clinicopathologic correlation.Zillmer DA et al

External Links

Citation

Salvatore Romeo ; Pancras CW Hogendoorn

Bone: Chondromyxoid fibroma

Atlas Genet Cytogenet Oncol Haematol. 2003-07-01

Online version: http://atlasgeneticsoncology.org/solid-tumor/5149/bone-chondromyxoid-fibroma