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A Preclinical Mouse Model of Osteosarcoma to Define the Extracellular Vesicle-mediated Communication Between Tumor and Mesenchymal Stem Cells
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Dermatomyofibroma-A rare mesenchymal tumor with maintained horripilation
Uwe Wollina1, Jacqueline Schönlebe2
1Department of Dermatology and Allergology, Academic Teaching Hospital Dresden, Dresden, Germany.
Dermatologic Therapy
|May 16, 2019
Summary
Dermatomyofibroma, a rare skin tumor, differs from dermatofibrosarcoma protuberans by lacking specific gene rearrangements. This case highlights maintained horripilation as a unique clinical feature.
Area of Science:
- Dermatopathology
- Oncology
- Genetics
Background:
- Dermatomyofibroma is a rare benign mesenchymal skin neoplasm.
- Distinguishing it from dermatofibrosarcoma protuberans (DFSP) is crucial due to malignant potential of DFSP.
- Both tumors share CD34-positive spindle cell morphology.
Observation:
- A case of axillary dermatomyofibroma in a 31-year-old woman is presented.
- A novel clinical observation of maintained horripilation (goosebumps) was noted.
- This contrasts with the typical absence of horripilation in DFSP.
Findings:
- Immunohistochemistry confirmed CD34 positivity in tumor cells.
- Crucially, genetic analysis revealed no COL1A1-PDGFB gene rearrangement, characteristic of DFSP.
- The presence of sustained horripilation is a newly described clinical sign.
Implications:
- This finding aids in differentiating benign dermatomyofibroma from malignant DFSP.
- Maintained horripilation may serve as a clinical diagnostic clue.
- Further research can explore the underlying mechanisms of this unique presentation.
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