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
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.
CHARACTERIZATION Radiographs Eccentric geographic lucency, well demarcated from the surrounding bone which has a rim of sclerosis
CHARACTERIZATION General Tumors range from 3-8 cm
Well circumscribed, solid, tan-gray
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
PROGNOSIS AND TREATMENT CHARACTERIZATION Treatment Complete removal
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Last Updated 8/20/2001
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