Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate.
| Autor: | Fiore M; Department of Surgery, Istituto Nazionale per lo studio e la cura dei Tumori, via Venezian 1, 20133 Milano, Italy., Miceli R, Mussi C, Lo Vullo S, Mariani L, Lozza L, Collini P, Olmi P, Casali PG, Gronchi A |
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| Jazyk: | angličtina |
| Zdroj: | Journal of clinical oncology : official journal of the American Society of Clinical Oncology [J Clin Oncol] 2005 Oct 20; Vol. 23 (30), pp. 7669-75. |
| DOI: | 10.1200/JCO.2005.02.5122 |
| Abstrakt: | Purpose: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) beta-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. |
| Databáze: | MEDLINE |
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