University Children's Hospital Muenster, Department of Pediatric Hematology and Oncology, 48149 Muenster, Germany.
Cancer. 2010 Jan 15;116(2):443-50. doi: 10.1002/cncr.24740.
The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES) was investigated.
We analyzed 120 patients registered into the European Ewing Tumor Working Initiative of National Groups (EURO-E.W.I.N.G. 99) trial at the trial center of Muenster from 1998 to 2006. Median age was 16.2 years. Local treatment of the primary tumor was surgery in 26 of 120 patients, surgery and radiotherapy in 21 patients, and definitive radiotherapy in 40 patients. For treatment of metastases, 6 of 120 patients received surgery; 9 patients, surgery and radiotherapy; and 33 patients, definitive radiotherapy. Forty-seven (39%) patients had local treatment of both the primary tumor and metastases, 41 (34%) patients of either the primary tumor or metastases, and 32 (27%) received no local therapy.
Event-free survival (EFS) at 3 years was 0.24 (95% CI, 0.16-0.33). Univariate analyses demonstrated the impact of local therapy given to the primary tumor: 3-year EFS was 0.25 with surgery, 0.47 with surgery and radiotherapy, 0.23 with radiotherapy, and 0.13 when no local therapy was administered (P < .001). Three-year EFS in PDMES was also influenced by the local treatment: surgery, 0.33; surgery and radiotherapy, 0.56; radiotherapy, 0.35; no local therapy, 0.16 (P = .003). Three-year EFS was 0.39 in patients who received local treatment of both primary tumor and PDMES, compared with 0.17 in patients with any local treatment of either primary tumor or PDMES and 0.14 in patients with no local therapy (P < .001). Multivariate analysis showed absence of local treatment to be the major risk factor (HR = 2.21; P = .027; n = 20).
Local therapy of involved sites is important for patients with PDMES and should complement systemic treatment whenever possible.
本研究旨在探讨局部治疗在原发性、播散性、多灶性尤文肉瘤（PDMES）患者中的作用。
我们分析了 1998 年至 2006 年期间在明斯特欧洲尤文肿瘤研究组（EURO-E.W.I.N.G. 99）试验中心登记的 120 名患者。中位年龄为 16.2 岁。26 例患者接受了原发肿瘤的手术治疗，21 例患者接受了手术联合放疗，40 例患者接受了根治性放疗。6 例患者接受了转移灶的手术治疗，9 例患者接受了手术联合放疗，33 例患者接受了根治性放疗。47 例（39%）患者同时接受了原发肿瘤和转移灶的局部治疗，41 例（34%）患者仅接受了原发肿瘤或转移灶的局部治疗，32 例（27%）患者未接受局部治疗。
3 年无事件生存率（EFS）为 0.24（95%CI，0.16-0.33）。单因素分析显示，原发肿瘤局部治疗的影响：手术治疗患者的 3 年 EFS 为 0.25，手术联合放疗为 0.47，放疗为 0.23，未行局部治疗为 0.13（P<.001）。PDMES 的 3 年 EFS 也受到局部治疗的影响：手术治疗为 0.33，手术联合放疗为 0.56，放疗为 0.35，未行局部治疗为 0.16（P=.003）。接受原发肿瘤和转移灶局部治疗的患者 3 年 EFS 为 0.39，而仅接受原发肿瘤或转移灶任何局部治疗的患者为 0.17，未行局部治疗的患者为 0.14（P<.001）。多因素分析显示，未行局部治疗是主要的危险因素（HR=2.21；P=0.027；n=20）。
局部治疗受累部位对 PDMES 患者很重要，应尽可能与全身治疗相补充。
