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Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report
Francois G Kamar1, Victor F Kairouz, Alain N Sabri
1Division of Hematology & Oncology, Clemenceau Medical Center, City Center Building, Suite 3 A, Avenue Nouvelle, P,O, Box 1076, Beirut, Jounieh, Lebanon. kamars@idm.net.lb.
Abstract:
DFSP is a locally invasive, slow-growing tumor of the subcutaneous tissue that rarely metastasizes but recurs frequently after surgical excision. We report herein a case of highly recurrent, locally invasive DFSP that failed both postoperative radiation therapy and complete trial of Imatinib, but was successfully treated with Sorafenib, which showed unprecedented response.
Insights
Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor that often recurs after surgery. This case highlights Sorafenib as a novel treatment for recurrent DFSP that resisted other therapies.
Area of Science:
- Oncology
- Dermatology
- Surgical Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, locally invasive, slow-growing soft tissue sarcoma.
- DFSP typically arises in the subcutaneous tissue and is characterized by frequent recurrences after surgical excision, despite its low metastatic potential.
Observation:
- A case of highly recurrent and locally invasive DFSP is presented.
- The patient's tumor demonstrated resistance to standard treatment modalities, including postoperative radiation therapy and imatinib.
Findings:
- Successful treatment of refractory, recurrent DFSP was achieved using Sorafenib.
- Sorafenib demonstrated an unprecedented and significant response in this challenging DFSP case.
Implications:
- Sorafenib represents a promising therapeutic option for advanced or refractory DFSP.
- This finding may guide future treatment strategies for patients with difficult-to-treat DFSP.
- Further research is warranted to explore the efficacy and mechanisms of Sorafenib in DFSP treatment.