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Updated: Aug 5, 2026
08:49
A 3D Organotypic Melanoma Spheroid Skin Model
Published on: May 18, 2018
Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior
1Peter MacCallum Cancer Centre, East Melbourne, Victoria, Australia. grant.mcarthur@petermac.org
Current Opinion in Oncology
|May 25, 2006
Summary
Dermatofibrosarcoma protuberans (DFSP) management relies on surgery for excellent local control. For unresectable or metastatic DFSP, targeted therapy inhibiting platelet-derived growth factor receptors shows promising results.
Area of Science:
- Oncology
- Dermatology
- Genetics
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade skin malignancy.
- Characterized by infiltrative growth and specific chromosomal translocations (COL1A1-PDGFB).
- Metastatic potential is low but present.
Purpose of the Study:
- Review current data on surgical management of DFSP.
- Examine novel molecularly targeted therapies for unresectable DFSP.
- Assess the role of platelet-derived growth factor (PDGF) receptor inhibition.
Main Methods:
- Literature review of recent studies on DFSP.
- Analysis of surgical outcomes and targeted therapy efficacy.
- Focus on COL1A1-PDGFB translocation and PDGF receptor inhibitors.
Main Results:
- Surgery achieves over 95% local control for DFSP.
- Imatinib (Gleevec), a PDGF receptor inhibitor, shows significant clinical responses in unresectable/metastatic DFSP.
- Complete responses observed in some patients treated with imatinib.
Conclusions:
- PDGFB gene translocation is key in DFSP pathogenesis.
- Targeted therapy with PDGF receptor inhibitors like imatinib is effective for advanced DFSP.
- While surgery is standard, targeted therapy offers a vital option for non-surgical candidates.
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