Harvard Radiation Oncology Program, Brigham and Women's Hospital, Boston, MA 02115, USA.
Int J Radiat Oncol Biol Phys. 2012 Jan 1;82(1):361-7. doi: 10.1016/j.ijrobp.2010.08.042. Epub 2010 Oct 15.
Myxofibrosarcoma (MFS) is a rare sarcoma with a predilection for multiple local recurrences (LR), for which 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 were treated at our institution. Data on clinicopathologic features, treatments, and patient outcomes were reviewed and analyzed.
Median age was 72.5 years (range, 42-96 years). Median tumor size was 7.5 cm, and 34 tumors (94%) were high grade. All patients underwent surgery at our institution, including re-resections in 20 patients (56%) after initial surgery elsewhere. Margins were microscopically positive in 9 patients (25%). RT was given to 28 patients (78%) pre - and/or postoperatively. After a median follow-up of 3.5 years (range, 0.4-12.4 years), 11 patients (31%) developed LR. There were no significant predictors for LR on univariate analysis, including margin status or use of RT. Limb salvage was ultimately achieved in only 5 of 11 LRs (45%) because of multiple subsequent LRs. Distant recurrence (DR) occurred in 6 patients (17%). Median and 4-year OS were 96 months and 65%, respectively. Seven patients (19%) died of tumor-related causes, 6 of whom had DRs. On univariate analysis, tumor size was associated with OS.
Despite aggressive surgery and RT, intermediate- and high-grade MFS are associated with a high rate of LR that adversely affects limb preservation. More aggressive local treatment strategies are necessary.
黏液纤维肉瘤（MFS）是一种罕见的肉瘤，多有多处局部复发（LR）的倾向，其最佳治疗方法尚未确定。我们回顾了我们的经验，以确定手术和放射治疗（RT）对复发模式、保肢和总生存（OS）的影响。
1995 年至 2005 年间，我们机构治疗了 36 例局部中-高级 MFS 患者。回顾了临床病理特征、治疗和患者结局的数据，并进行了分析。
中位年龄为 72.5 岁（范围，42-96 岁）。中位肿瘤大小为 7.5cm，34 个肿瘤（94%）为高级别。所有患者均在我们机构接受了手术，包括 20 例（56%）患者在最初手术的其他地方进行了再切除。9 例（25%）患者的切缘显微镜下阳性。28 例（78%）患者在术前和/或术后接受了 RT。中位随访 3.5 年后（范围，0.4-12.4 年），11 例（31%）患者发生 LR。单因素分析未发现 LR 的显著预测因素，包括切缘状态或 RT 的使用。由于随后发生多次 LR，仅有 11 例 LR 中的 5 例（45%）最终实现了保肢。6 例（17%）患者发生远处复发（DR）。中位和 4 年 OS 分别为 96 个月和 65%。7 例（19%）患者死于肿瘤相关原因，其中 6 例发生 DR。单因素分析显示，肿瘤大小与 OS 相关。
尽管进行了积极的手术和 RT，中-高级 MFS 仍与 LR 发生率高相关，这对保肢产生了不利影响。需要更积极的局部治疗策略。
