Department of Hematology/Oncology, Hospital for Sick Children, Toronto, Ontario, Canada.
Cancer. 2010 Jul 1;116(13):3189-94. doi: 10.1002/cncr.25144.
As Ewing sarcoma (EWS) can affect children and adults, these patients can be treated at either a pediatric or an adult institution. This study investigated whether differences in therapeutic strategy undertaken in pediatric and adult specialty sarcoma centers correlated with clinical outcome.
Data from patients with localized EWS treated between 1990 and 2005 at tertiary care pediatric and adult institutions were reviewed.
Fifty-three patients (24 adult and 29 pediatric) were treated. Pediatric patients received a median of 16 cycles of chemotherapy comprised of doxorubicin, vincristine, cyclophosphamide, ifosfamide, and etoposide. Adult patients received a median of 10 cycles of treatment, and a significantly lower total cumulative dose of ifosfamide and cyclophosphamide (P < .0001). There was no difference noted with regard to the total dose of doxorubicin, or in the type of local therapy offered (surgery or radiotherapy, vs both). However, local therapy occurred earlier in pediatric patients compared with adults (3.7 months vs 7.4 months; P = .0003). The 3-year event-free survival (EFS) rate in pediatric and adult patients was 70% +/- 9% and 43% +/- 13% (P = 0.1), respectively. The 3-year overall survival rate was 81% +/- 7.7% and 59% +/- 12% (P = .02) for pediatric and adult patients, respectively. Factors found to be significantly associated with EFS on univariate analysis included pelvic site, cyclophosphamide dose, and time to local therapy. On multivariate analysis, only pelvic disease (hazard ratio [HR] 4.26; P = .018) and time to local therapy (HR, 1.19; P = .002) were found to be significant.
Adults with localized EWS have an inferior outcome compared with pediatric patients. This difference may be related to lower doses of alkylating agents and the timing of local therapy.
尤文肉瘤（EWS）可影响儿童和成人，这些患者可在儿科或成人专业肉瘤中心接受治疗。本研究旨在调查儿科和成人专科肉瘤中心采用的治疗策略差异是否与临床结局相关。
回顾了 1990 年至 2005 年间在三级护理儿科和成人机构接受治疗的局限性 EWS 患者的数据。
53 例患者（24 例成人和 29 例儿科）接受了治疗。儿科患者接受了中位数为 16 个周期的化疗，包括阿霉素、长春新碱、环磷酰胺、异环磷酰胺和依托泊苷。成人患者接受了中位数为 10 个周期的治疗，并且异环磷酰胺和环磷酰胺的总累积剂量明显较低（P <.0001）。在阿霉素的总剂量或所提供的局部治疗类型（手术或放疗，或两者兼有）方面，两组之间没有差异。然而，与成人患者相比，儿科患者的局部治疗更早开始（3.7 个月 vs 7.4 个月；P =.0003）。儿科和成年患者的 3 年无事件生存率（EFS）分别为 70% +/- 9%和 43% +/- 13%（P = 0.1）。3 年总生存率分别为 81% +/- 7.7%和 59% +/- 12%（P =.02）。单因素分析发现，与 EFS 显著相关的因素包括骨盆部位、环磷酰胺剂量和局部治疗时间。多因素分析显示，仅骨盆疾病（危险比 [HR] 4.26；P =.018）和局部治疗时间（HR，1.19；P =.002）是显著相关的因素。
与儿科患者相比，局限性 EWS 成人患者的预后较差。这种差异可能与烷化剂剂量较低和局部治疗时间有关。
