背景:青少年和青壮年(AYAs)尤文肉瘤的预后比儿童差。基于人群的生存评估通过重要的临床因素对结果进行分层,然而,limited.这项荷兰人口研究综合比较了30年来儿童和AYAs与尤因肉瘤的存活率,考虑到诊断期,组织起源,肿瘤部位,疾病阶段。
方法:所有儿童的数据(0-17岁,N=463)和AYAs(18-39岁,N=379)从荷兰癌症登记处收集1990-2018年间在荷兰诊断为尤因肉瘤,随访至2023年2月。使用队列方法计算5年相对生存率。通过泊松回归进行多变量分析。
结果:尤因肉瘤患儿的5年相对生存率明显高于AYAs(65%vs.44%)。在2010-2018年,儿童生存率呈上升趋势,达到70%,AYAs为53%。尤因骨肉瘤和骨外尤因肉瘤的结果相似。对于大多数肿瘤部位,无论疾病阶段如何,AYAs的预后都比儿童差。生存概率为60%vs.局部疾病占78%,20%与33%为转移性疾病。多元回归分析,调整了随访时间,诊断期,性别,疾病阶段,和肿瘤部位,确认与儿童相比,AYA的超额死亡率增加(超额HR:1.7,95%CI:1.3-2.1)。
结论:尽管自1990年代以来生存有所改善,AYAs与荷兰的尤因肉瘤的情况仍然比儿童差得多。无论起源组织如何,这种生存差异都存在,肿瘤部位,疾病阶段。
BACKGROUND: Adolescents and young adults (AYAs) with Ewing sarcoma have a worse prognosis than children. Population-based survival evaluations stratifying findings by important clinical factors are, however, limited. This Dutch population study comprehensively compared survival of children and AYAs with Ewing sarcoma over three decades considering diagnostic period, tissue of origin, tumor site, and disease stage.
METHODS: Data on all children (0-17 years, N = 463) and AYAs (18-39 years, N = 379) diagnosed with Ewing sarcoma in the Netherlands between 1990-2018 were collected from the Netherlands Cancer Registry with follow-up until February 2023. Five-year relative survival was calculated using the cohort method. Multivariable analyses were conducted through Poisson regression.
RESULTS: Children with Ewing sarcoma had a significantly higher 5-year relative survival than AYAs (65 % vs. 44 %). An increasing trend in survival was noted reaching 70 % in children and 53 % in AYAs in 2010-2018. Results were similar for Ewing bone sarcoma and extraosseous Ewing sarcoma. AYAs had a poorer prognosis than children for most tumor sites and regardless of disease stage. Survival probabilities were 60 % vs. 78 % for localized disease and 20 % vs. 33 % for metastatic disease. Multivariable-regression analysis, adjusted for follow-up time, diagnostic period, sex, disease stage, and tumor site, confirmed increased excess mortality among AYAs compared with children (excess HR: 1.7, 95 % CI: 1.3-2.1).
CONCLUSIONS: Despite survival improvements since the 1990s, AYAs with Ewing sarcoma in the Netherlands continue to fare considerably worse than children. This survival disparity was present irrespective of tissue of origin, tumor site, and disease stage.