Dermatofibrosarcoma protuberans: a clinicopathological study of 20 cases.

Area of Science:

• Dermatology
• Oncology
• Pathology

Background:

• Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin neoplasm.
• DFSP presents diagnostic challenges due to its varied clinical and histological appearance.
• Understanding DFSP's characteristics is crucial for effective management and treatment.

Purpose of the Study:

• To review the clinical and histological data of 20 DFSP cases.
• To analyze morphologic variants and immunohistochemical findings in DFSP.
• To evaluate treatment outcomes and recurrence rates for DFSP.

Main Methods:

• Retrospective review of 20 DFSP cases from two Lisbon dermatology centers (1978-1998).
• Analysis of clinical presentation, histopathology (including variants), and immunohistochemistry (CD34, FXIIIa).
• Comparison of treatment outcomes between wide local excision and Mohs micrographic surgery.

Main Results:

• The median age at diagnosis was 51 years, with the trunk being the most frequent location.
• All cases showed the characteristic storiform pattern; fibrosarcomatous changes were noted in three.
• CD34 expression was observed in 18/20 specimens; CD34 and FXIIIa were key for differential diagnosis.
• Recurrence rate was 15% regardless of treatment (wide excision vs. Mohs surgery).

Conclusions:

• DFSP requires careful histological and immunohistochemical evaluation for accurate diagnosis.
• Both wide local excision and Mohs micrographic surgery showed similar local recurrence rates in this series.
• Further research into optimal surgical margins and long-term outcomes for DFSP is warranted.