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Background: Dermatofibrosarcoma protuberans is a rare low-grade sarcoma of the skin with a tendency to recur locally after inadequate excision. Treatment has traditionally been wide excision with a 2- to 3-cm gross margin. Because of the variable results presented in mainly retrospective reports, it has been queried whether local control can be as good with conventional surgery as with micrographic surgery. Methods: Forty patients with dermatofibrosarcoma protuberans treated by surgical excision were operated on at our center from 1987 to 2001. Data were recorded prospectively. Twenty-seven patients presented with a primary tumor and 13 with a locally recurrent tumor primarily operated on elsewhere. Gross and histologic margins were studied in detail. Results: At a mean follow-up of 40 months, there were no recurrences. Thirty-four patients required single, five patients two, and one patient three operations before the margins were adequate (mean, 1.2 stages per patient). Twenty-three patients (58 percent) needed reconstructions. Tumor-free margins were obtained in 39 patients. The average thickness of surgical gross margins was 3.1 cm; histologically defined margins averaged 1.6 cm. Conclusions: Good local control can be achieved with wide surgery. Histologic tumor-free margins differ greatly from gross margins and are difficult to assess clinically and macroscopically. Careful postoperative histologic examination with margins measured in millimeters should be carried out to define the adequacy of excision in all directions. On average, a 1.6-cm histologic margin was adequate for complete local control. Most patients can be operated on in one stage. Reconstructions are often needed.

The objective of this retrospective follow-up study was to evaluate the outcome of patients with soft-tissue sarcoma treated by the authors' protocol, which consists of a selective combination of conservative surgery and radiotherapy. Patients who relapsed were especially evaluated to improve treatment results. The authors examined 80 patients with local soft-tissue sarcoma in the upper extremity referred to their multidisciplinary group. Fifteen patients were referred for first or subsequent local recurrence, and 65 patients were treated for primary tumor. The goal of treatment was local control and preservation of a functional limb. Wide excision was attempted. If the margin was less than 2.5 cm, postoperative radiotherapy was administered. Eighty-five percent of the patients were treated by limb salvage. Thirty patients needed reconstructive procedures such as pedicled (20 patients) or free flaps (10 patients). No free flaps were lost. The 5-year disease-specific overall survival rate was 75 percent, the local recurrence-free survival rate was 79 percent, and the metastasis-free survival rate was 68 percent. In univariate analysis, prognostic factors for local recurrence were extracompartmental site; for development of metastases, large size and extracompartmental site; and for decreased disease-specific overall survival, large size and extracompartmental site. Intramuscular, cutaneous, and subcutaneous tumors had a 5-year local control rate of 100 percent, and extracompartmental tumors had a local control rate of 69 percent. Extracompartmental tumors clearly have the worst prognosis and should be the main target for improving treatment strategies. After exclusion of patients with inadequate treatment according to the authors' protocol, the local control rate at 5 years was 90 percent. Strict adherence to treatment protocol should be practiced.