Management of dermatofibrosarcoma protuberans with fibrosarcomatous transformation: an evidence‐based review of the literature

Abstract

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.

Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline

“…Only two guidelines were found and their latest revisions have been published in 2012 [1] or 2013 [2]. We found only one relevant systematic review on the efficacy of MMS in the treatment of DFSP [3] and one on the management of dermatofibrosarcoma protuberans with fibrosarcomatous transformation [4]. No important differences were found between German and US guidelines.…”

Neoadjuvant Imatinib in Advanced Primary or Locally Recurrent Dermatofibrosarcoma Protuberans: A Multicenter Phase II DeCOG Trial with Long-term Follow-up

“…Under the premise of tumor-free surgical margins, the rates of local recurrence and metastasis are low (5,6), rendering the main therapeutic efforts focused on the primary tumor. This situation is different in fibrosarcomatous DFSP (DFSP-FS), which presents several characteristics of high-grade sarcoma and accordingly shows an increased risk of relapse as well as a worse prognosis (7).…”

Imaging Findings of Head and Neck Dermatofibrosarcoma Protuberans

“…[12][13][14]35 Most local recurrences occur within 3 years 11,12,35 ; only 30% occur after 5 years. 32 The lungs are the most common site of distant metastasis, 12,30,36,37 and metastasis has been reported to occur in 5-6% of cases. 13,14,34,35,38,39 Differential diagnoses of other cutaneous neoplasms that could be confused with DFSP include dermatofibroma, epidermal cyst, peripheral nerve sheath tumor, nodular fasciitis, cavernous hemangioma, liposarcoma, nodular type of melanoma, and mycosis fungoides.…”