Dermatofibrosarcoma protuberans: a case series of 16 patients treated in a single institution with literature review.

摘要

BACKGROUND: Dermatofibrosarcoma protuberans is an uncommon skin tumour with a low to intermediate-grade of malignancy, characterized by progressive growth and a propensity for local recurrence. PATIENTS AND METHODS: A retrospective study of a series of 16 consecutive patients with dermatofibrosarcoma protuberans who were treated in the host Institution over the last seven years was performed, with special emphasis on the outcome and disease-free interval, as well as recurrence rate over a mean follow-up period of 43.65 months. RESULTS: The clinicopathological features and results were reviewed. The primary treatment consisted of wide local excision with or without radiotherapy on 13 patients with primary and 3 with recurrent disease, and all patients remained free of disease recurrence during the mean follow-up period. CONCLUSION: The results of this study and a review of the literature support the notion that aggressive, wide surgical resection with disease-free margins, with or without radiotherapy decreases local recurrences and offers an excellent probability of cure. The accumulated data also confirm that all patients with dermatofibrosarcoma protuberans should be followed up for an extended period, beyond the usual recommended 5-year follow-up, because late recurrences may occur.