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

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. Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma that was first described by Taylor in 1890 (1, 2), clinically was classified by Darier and Ferrand (3) and later named by Hoffman (4). It is a rare skin tumour that clinically often masquerades as a benign, indolent tumour, but microscopically it extends far beyond assessed clinically margins, and spreads locally in the dermis, subcutaneous tissue and muscles (5). Approximately 85-90% of all DFSPs are low-grade lesions while the rest contain a high grade fibrosarcomatous component and are considered to be intermediate-grade neoplasms (6). The goal of this retrospective review of 16 patients who underwent wide local excision with or without radiotherapy for the treatment of DFSP tumours was to expand on the clinicopathological characterization and treatment strategy of this unusual neoplasm. Awareness of this entity is important for a prompt diagnosis and a proper management of the disease, preventing both over- and undertreatment of this low to intermediate-grade malignancy.