背景:轴向骨骼的尤因肉瘤对临床医生来说是一个显著的挑战,因为它们具有积极的表现和阻塞神经血管结构的倾向;然而,关于儿童轴性肿瘤的数据很少。这项研究是第一个基于人群的分析,评估轴向尤文肉瘤的治疗方案及其对癌症特异性生存率和总生存率(OS)的影响。
方法:收集了2004年至2019年的所有1至24岁患者的数据,流行病学,和结束结果(SEER)数据库。主要组包括盆腔肿瘤,胸部肿瘤,和脊椎肿瘤.卡方检验和Kaplan-Meier检验用于评估人口统计学变量之间的关联,临床和治疗特点,和病人的生存。
结果:盆腔肿瘤最常见,49.7%接受化疗/放疗。脊椎肿瘤是最不常见的,56.7%接受化疗/手术/放疗。53.5%的胸部肿瘤接受化疗/手术。手术最常见于胸部肿瘤(80.2%),罕见于盆腔肿瘤(38.9%)。放射治疗最常见的是椎体肿瘤(83.6%),最不常见的是胸部肿瘤(36.0%)。盆腔肿瘤表现出最低的OS(1年,5年,十年OS:96%,70%,和59%),其次是胸部肿瘤(1年,5年,10年OS:97%,79%,和66%)和椎体肿瘤(1年,5年,10年OS:92%,77%,和68%)。
结论:这项研究强调了早期发现和基于化疗的多模式治疗在儿童人群中轴Ewing肉瘤治疗中的重要性。对于胸部肿瘤患者,在5到10年间观察到了相对较大的OS下降,与1973年至2011年的类似SEER队列相比,该队列的10年OS没有改善。尽管越来越多的研究支持确定性放射治疗,值得注意的一部分骨盆尤文肉瘤患者没有接受放射治疗,代表了这个人口的未满足需求。
BACKGROUND: Ewing sarcomas of the axial skeleton represent a notable challenge for clinicians because of their aggressive presentation and tendency to obstruct neurovascular structures; however, little data exist regarding axial tumors in children. This study is the first population-based analysis assessing treatment regimens for axial Ewing sarcomas and their effects on cancer-specific survival and overall survival (OS).
METHODS: Data from 2004 to 2019 were collected for all patients aged 1 to 24 years from the Surveillance, Epidemiology, and End Results (SEER) database. Primary groups included pelvic tumors, thoracic tumors, and vertebral tumors. Chi-squared and Kaplan-Meier tests were used to assess associations between demographic variables, clinical and treatment characteristics, and patient survival.
RESULTS: Pelvic tumors were most common, and 49.7% received chemotherapy/radiation. Vertebral tumors were least common, and 56.7% received chemotherapy/surgery/radiation. 53.5% of thoracic tumors received chemotherapy/surgery. Surgery was most common for thoracic tumors (80.2%) and rare for pelvic tumors (38.9%). Radiation therapy was most common for vertebral tumors (83.6%) and least common for thoracic tumors (36.0%). Pelvic tumors exhibited the lowest OS (1-year, 5-year, and 10-year OS: 96%, 70%, and 59%), followed by thoracic tumors (1-year, 5-year, and 10-year OS: 97%, 79%, and 66%) and vertebral tumors (1-year, 5-year, and 10-year OS: 92%, 77%, and 68%).
CONCLUSIONS: This study underpins the importance of both early detection and chemotherapy-based multimodal therapy in the treatment of axial Ewing sarcoma in a pediatric population. A comparatively large decline in OS was observed between 5 and 10 years for patients with thoracic tumors, and this cohort\'s 10-year OS has not improved when compared with a similar SEER cohort from 1973 to 2011. Despite a growing body of research supporting definitive radiation therapy, a notable portion of patients with pelvic Ewing sarcoma did not receive radiation, representing an unmet need for this population.