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Related Experiment Video

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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

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Desmoplastic fibroma with malignant transformation.

Hye Sook Min¹, Hyun Gyui Kang, Joo-Hyuk Lee

¹Department of Pathology, National Cancer Center, Goyang, Gyoung-Gi, Korea.

Annals of Diagnostic Pathology

|February 4, 2010

Summary

This study reports a rare case of desmoplastic fibroma (DF) in bone with unusual histology. The findings suggest potential malignant transformation of DF, highlighting the need for further research into this aggressive bone neoplasm.

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Area of Science:

Bone oncology
Skeletal pathology
Neoplastic disease of bone

Background:

Desmoplastic fibroma (DF) is a rare, locally aggressive bone neoplasm.
DFs typically present as infiltrative lesions.
Understanding DF variants is crucial for diagnosis and treatment.

Observation:

A 41-year-old female presented with a left distal femur lesion.
Radiography revealed an osteolytic lesion; MRI showed a well-demarcated mass.
Pathology demonstrated a DF with mixed myxofibrosarcomatous and malignant fibrous histiocytoma components.

Findings:

The tumor exhibited bland spindle cells in a collagenous stroma.
Histologically distinct areas were admixed within the neoplasm.
Comparative genomic hybridization revealed specific genetic alterations correlating with histologic features.

Implications:

This case may represent malignant transformation of desmoplastic fibroma.
Unusual histology and genetic events in DF warrant further investigation.
Findings contribute to the understanding of rare bone tumor behavior.