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[Dermatofibrosarcoma protuberans. About 18 cases]
I Zaraa1, E Cherif, L Ferchichi
1Service de dermatologie, Hôpital La Rabta, Tunis.
Abstract:
Dermatofibrosarcoma protuberans (DFSP) is a rare mesenchyma skin tumor. It is characterized by a slow growth with a high rate of recurrence but limited potential for metastasis. We report the result of a retrospective study of 18 cases of DFSP collected over 16 years in the dermatology department of La Rabta Hospital. The mean age was 32 years with a sex ratio of 1.57. The site of predilection was the trunk (77.5%). Histological features were typical in all cases with positivity for CD34 in 14 cases. Treatment was surgical in 13 cases. DFSP is a tumor of intermediate malignancy, which can have aggressive course. This tumor is best treated with surgery in which large excision are necessary to reduce the risk of recurrence.
Insights
Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor. Surgical excision is crucial for managing this intermediate malignancy to minimize recurrence risk.
Area of Science:
- Dermatology
- Oncology
- Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing cutaneous mesenchymal tumor.
- DFSP exhibits a high recurrence rate but limited metastatic potential.
- Understanding DFSP epidemiology and treatment is vital for patient outcomes.
Observation:
- A retrospective study analyzed 18 DFSP cases over 16 years.
- The mean patient age was 32 years, with a 1.57 male-to-female ratio.
- The trunk was the most common site (77.5%), and CD34 positivity was observed in 14 cases.
Findings:
- Histological features were typical in all DFSP cases.
- Surgical treatment was performed in 13 out of 18 cases.
- DFSP is an intermediate malignancy with potential for aggressive behavior.
Implications:
- Wide surgical excision is the recommended treatment for DFSP.
- Effective management strategies are essential to reduce DFSP recurrence.
- Further research into DFSP can improve therapeutic approaches.
