Background

This very rare benign bone tumor has a varied morphology and may histologically mimic a sarcoma. These tumors occur in younger patients and arise within the metaphysis of long tubular bones. However, these tumors have a widespread distribution. Dull achy pain is the most common symptom, localized to the tumor site.

AGE RANGE-MEDIAN	2-3rd decades
SEX (M:F)	M

 

PATHOGENESIS

CHARACTERIZATION

Cell Biology and Matrix Biochemistry of Chondromyxoid Fibroma Stephan Söder, MD, Carrie Inwards, MD, Susanna Müller, MD, Thomas Kirchner, MD, and Thomas Aigner, MD

Am J Clin Pathol 2001;116:271-277 Abstract quote We studied matrix composition and gene expression pattern in chondromyxoid fibromas on the protein and the messenger RNA levels. We could clearly identify focal chondrocytic differentiation within chondromyxoid fibroma by the expression and deposition of type II collagen, which is a marker of chondrocytic cell differentiation. We also were able to show expression of collagen types I, III, and VI in the neoplasm. The major tumor portion was, however, characterized by the presence of hydrated proteoglycans and only minor amounts of collagens, a matrix composition responsible for the myxoid matrix appearance of most parts of these neoplasms. By analyzing cytoprotein expression, we found S-100 protein restricted to cells of the chondroid areas, suggesting S-100 protein staining to be of little help as a positive diagnostic marker for chondromyxoid fibroma. Our data show a specific matrix composition of chondromyxoid fibroma, not previously found in other mesenchymal neoplasms, including chondroblastoma, osteochondroma, enchondroma, and chondrosarcoma. This justifies chondromyxoid fibroma as a specific neoplastic entity, both clinically and biologically.

 

LABORATORY/RADIOLOGIC/ OTHER TESTS	CHARACTERIZATION
Radiographs	Eccentric geographic lucency, well demarcated from the surrounding bone which has a rim of sclerosis

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
General	Tumors range from 3-8 cm Well circumscribed, solid, tan-gray
VARIANTS

 

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Extensive myxomatous areas surrounded in a lobular pattern by a more cellular rim Areas of putative chondroid and, less often, fibrous differentiation can be found
VARIANTS

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
Treatment	Complete removal

Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.

Bone Tumors

Last Updated 8/20/2001

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