Wide excision with margins ≥2 cm significantly reduced local recurrence compared to local excision without defined margins in patients with transformed dermatofibrosarcoma protuberans (P=0.01).
Does wide excision with margins ≥2 cm reduce local recurrence in patients with transformed dermatofibrosarcoma protuberans compared to local excision without defined margins?
Wide excision with margins ≥2 cm represents the optimal surgical treatment for transformed dermatofibrosarcoma protuberans to prevent local recurrence and subsequent metastasis.
p-value: p=0.01
Fibrosarcomatous transformation represents a rare event in dermatofibrosarcoma protuberans (DFSP) with unpredictable biological behaviour. No guidelines for the adequate treatment of patients with this rare neoplasm have been published. Herein, we present a comprehensive review of the literature comprising 157 patients with transformed DFSP focussing on surgical and adjuvant treatment modalities for this tumour. In the cohort examined, local recurrence occurred in 36% of cases and was significantly lower in patients treated by wide excision with margins ≥2 cm when compared with those treated with local excision without defined margins (P = 0.01). Consistently, negative margin status was associated with a lower recurrence rate when compared with positive or unknown margin status (P = 0.01). Distant metastases were detected in 13% of patients, which is significantly higher when compared with ordinary dermatofibrosarcoma protuberans. Systemic dissemination was preceded by local recurrence in 81% of cases, and is therefore strongly associated with tumour recurrence (P ≤ 0.001). The present data confirm that wide excision with margins ≥ 2 cm represent the gold standard in the treatment of transformed dermatofibrosarcoma protuberans, and prevents recurrence as well as metastasis. When R0-resection is not feasible, adjuvant radiation should be considered for cases with incomplete resection or unknown surgical margins. Irresectable or metastatic transformed DFSP harbouring the COL1A1-PDGFB fusion gene should be treated with imatinib in the palliative setting or as an adjunctive treatment before surgery, although responses may be short-lasting.
Voth et al. (Sat,) conducted a review in Dermatofibrosarcoma protuberans with fibrosarcomatous transformation (n=157). Wide excision with margins ≥2 cm vs. Local excision without defined margins was evaluated on Local recurrence (p=0.01). Wide excision with margins ≥2 cm significantly reduced local recurrence compared to local excision without defined margins in patients with transformed dermatofibrosarcoma protuberans (P=0.01).