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Dermatofibrosarcoma Protuberans Treated at a Single Institution: A Surgical Disease With a High Cure Rate

From the Department of Surgery, Istituto Nazionale per lo studio e la cura dei Tumori, and the Unit of Medical Statistics and Biometry, Istituto Nazionale per lo studio e la cura dei Tumori, and the Department of Radiation Oncology, Istituto Nazionale per lo studio e la cura dei Tumori, and the Department of Pathology, Istituto Nazionale per lo studio e la cura dei Tumori, and the Department of Cancer Medicine, Istituto Nazionale per lo studio e la cura dei Tumori, Milano, Italy

Address reprint requests to Alessandro Gronchi, MD, Department of Surgery, Istituto Nazionale per lo studio e la cura dei Tumori, via Venezian 1, 20133 Milano, Italy; e-mail: alessandro.gronchi{at}istitutotumori.mi.it

PURPOSE: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) ß-chain from gene rearrangement as a key pathogenetic factor, now susceptible of molecular-targeted therapy. The aim of this retrospective analysis was to explore the outcome of patients with primary or recurrent DFSP.

PATIENTS AND METHODS: Two hundred eighteen patients surgically treated at the Istituto Nazionale per lo studio e la cura dei Tumori (Milan, Italy) over 20 years were reviewed. Local relapse, distant metastasis, and survival were studied.

RESULTS: One hundred thirty-six patients (62.4%) presented with a primary DFSP, while 82 patients (37.6%) had a recurrent disease. In the primary group, margins were microscopically positive in 11.8%, while in the recurrent group they were positive in 14.6% (P =.613). In the primary group, patients undergoing re-excision after inadequate previous surgery had residual disease in 62% of cases. Reconstructive surgery was needed in 30%, significantly more frequently in patients with a recurrence or a head and neck tumor. The crude cumulative incidence of local relapses was 4% at 10 years, and 2% at 10 years for distant metastases. No significant difference was found between primary and recurrent patients, as well as between positive and negative margins.

CONCLUSION: This being one of the largest mono-institutional series of DFSP, we confirm that long-term outcome is excellent, in terms of both local and distant control, after a wide excision with negative margins. Reconstructive surgery is often needed. Novel medical therapies will be of use in a limited subgroup of patients.

Authors’ disclosures of potential conflicts of interest are found at the end of this article.

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