Krakorova Dagmar Adamkova, Kubackova Katerina, Dusek Ladislav, Tomas Tomas, Janicek Pavel, Tucek Stepan, Prausova Jana, Kiss Igor, Zambo Iva
Clinic of Comprehensive Cancer Care of Masaryk Memorial Cancer Institute, Brno, Czech Republic.
Second Faculty of Medicine, Charles University in Prague, Prague, Czech Republic.
Pathol Oncol Res. 2018 Jul;24(3):623-630. doi: 10.1007/s12253-017-0291-6. Epub 2017 Aug 12.
Ewing sarcoma (ES) is an exceptionally rare tumor in adults. Data regarding outcomes of adult patients with ES and experiences with age-adapted therapeutic strategies are very limited. The aim of this study was to evaluate prognostic factors and clinical outcome in a cohort of adult patients treated according to pediatric protocols in the Czech Republic. The records of 58 adult ES patients diagnosed between 2002 and 2013 were reviewed and factors relevant to prognosis and survival were analyzed. The median age of study cohort was 29 years (range, 18-59). The most frequent location was axial (36.2%), followed by involvement of extraskeletal tissues (34.5%) and bones of the extremities (29.3%). Twenty-eight (48.3%) patients had metastatic disease. In cases with localized ES, the 5-year overall survival (OS) was 76.5%. Using the log-rank test, the presence of metastasis at diagnosis, local treatment without surgery and a failure to achieve complete remission were associated with significantly shorter survival. In a multivariate Cox proportional hazard analysis, the achievement of complete remission was an independent predictor of patients's survival time. Outcomes of adults with localized ES treated according to multimodal pediatric protocols are similar to children. The achievement of complete remission is an independent predictor of survival time in ES patients. Severe hematological toxicity is foreseeable and manageable. Prognosis of patients with metastases or progression remains dismal.
尤因肉瘤（ES）在成人中是一种极其罕见的肿瘤。关于成年ES患者的预后数据以及适合年龄的治疗策略的经验非常有限。本研究的目的是评估在捷克共和国按照儿童方案治疗的成年患者队列中的预后因素和临床结局。回顾了2002年至2013年间诊断的58例成年ES患者的记录，并分析了与预后和生存相关的因素。研究队列的中位年龄为29岁（范围18 - 59岁）。最常见的部位是轴位（36.2%），其次是骨骼外组织受累（34.5%）和四肢骨骼（29.3%）。28例（48.3%）患者有转移性疾病。在局限性ES病例中，5年总生存率（OS）为76.5%。使用对数秩检验，诊断时存在转移、未进行手术的局部治疗以及未实现完全缓解与显著缩短的生存期相关。在多变量Cox比例风险分析中，实现完全缓解是患者生存时间的独立预测因素。按照多模式儿童方案治疗的局限性ES成年患者的结局与儿童相似。实现完全缓解是ES患者生存时间的独立预测因素。严重血液学毒性是可预见且可管理的。有转移或病情进展患者的预后仍然很差。
