MFS is a rare sarcoma with a predilection for multiple local recurrences (LR). In the absence of any large published series, optimal treatment has not been defined. We reviewed our experience to determine the impact of surgery and radiation therapy (RT) on pattern of recurrence, limb salvage, and overall survival (OS). Between 1995 and 2005, 36 patients with localized intermediate or high-grade MFS received treatment at Dana-Farber/Brigham and Women's Hospital Cancer Center (DFBWHCC). Data on clinicopathological features, treatments, and pt outcomes were reviewed. LR and OS rates were estimated using the Kaplan-Meier method. Comparisons of the difference in distributions were assessed by the log–rank test. Median age was 72.5 years (range, 42–96). Tumors were of high (n = 34) or intermediate (n = 2) grade. Median size was 7.5 cm (range, 0.7–22). Twelve patients (33%) had Stage II and 24 (67%) had Stage III disease. Disease sites were extremities (n = 32) and trunk (n = 4). All patients underwent surgery as part of initial treatment: 20 (56%) had re-resection at DFBWHCC after open biopsy or marginal resections elsewhere. One pt (3%) had amputation, the remainder had radical (n = 27, 75%) or wide (n = 8, 22%) excision. Final margins were microscopically positive in 9 patients (25%). RT was given to 28 patients (78%) either pre-operatively (n = 5; median, 50 Gy), postoperatively (n = 19; median, 62.5 Gy), or both (n = 4; median total, 64 Gy). Among the 8 patients who did not receive RT, 5 had negative surgical margins (mean, 1.5 cm; range, 0.5–3.5 cm), 2 had no residual tumor on re-excision, and 1 developed rapid LR before planned postoperative RT. One pt with a 22-cm MFS received preoperative chemotherapy (CT). No patients received adjuvant CT. After a median follow-up of 3.5 years (range, 0.4–12.4), 11 patients (30%) developed LR. Among those, median time to first LR was 21 mos (range, 6–48). The 4- year actuarial LR rate was 40%. Limb salvage was ultimately achieved for only 4 of 11 (36%) due to multiple LRs. The median number of LRs was 1 (range, 1–4). There were no significant predictors for LR on univariate analysis. Crude LR rates for patients treated with and without RT were 36% and 12%, respectively (p = NS); the rates for patients with positive and negative margins were 44% and 26%, (p = NS), respectively. Distant recurrence (DR) occurred in 6 patients (17%), most commonly in lung (n = 5). Among those 6 patients, median time to first DR was 21 mos (range, 4.8–97). Four of 6 DRs (67%) developed without any LR. Median OS was 96 mos and 4- year actuarial OS was 65%. Seven patients (20%) had tumor-related deaths, 6 of whom had DR. Despite aggressive surgery and RT, intermediate and high-grade MFS has a high rate of LR which adversely impacts limb preservation. More aggressive local treatment strategies are necessary.
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