title: 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.
authors.affiliation: Department of Dermatology and Allergology, Center of Integrated Oncology Cologne Bonn, University of Bonn, Bonn, Germany. harald.voth@ukb.uni-bonn.de
authors.forename: H
authors.initials: H
authors.lastname: Voth
authors.forename: J
authors.initials: J
authors.lastname: Landsberg
authors.forename: T
authors.initials: T
authors.lastname: Hinz
authors.forename: J
authors.initials: J
authors.lastname: Wenzel
authors.forename: T
authors.initials: T
authors.lastname: Bieber
authors.forename: G
authors.initials: G
authors.lastname: Reinhard
authors.forename: T
authors.initials: T
authors.lastname: Höller
authors.forename: C-M
authors.initials: CM
authors.lastname: Wendtner
authors.forename: M-H
authors.initials: MH
authors.lastname: Schmid-Wendtner
mesh_terms: D018223:Dermatofibrosarcoma / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D019317:Evidence-Based Medicine; D005354:Fibrosarcoma / Q000473:pathology*; D006801:Humans