背景:尤因肉瘤是一种侵袭性恶性肿瘤,主要影响儿童和青少年。关于治疗实践的研究有限,临床课程,和成年人的生存。
方法:对不列颠哥伦比亚省所有成人(>18岁)和儿童(≤18岁)的尤因肉瘤进行多机构回顾性队列研究,加拿大2000年1月1日至2018年12月31日。
结果:一百七个人(66名成年人,41名儿童)被纳入分析。5年OS在成人中为58%,在儿童中为75%。对于患有局部疾病的人来说,5年OS在成人中为74%,在儿童中为84%。成人状态与受损的PFS(HR,1.8;95%CI,1.0-3.1,p=0.04)和OS(HR,1.8;95%CI,0.9-3.5;p=0.088)。Charlson合并症指数(CCI)≥3与成人和儿童的生存受损有关(HR,3.9,95%CI,2.0-7.5;p=<0.001);基线CCI在组间没有显着差异。大多数成年人(61/66;92%)和所有儿童(41/41;100%)接受了全身治疗,平均治疗线没有显着差异,治疗方式或药物。大多数儿童接受间隔压缩化疗(35/41;85%),而成人(19/61;29%;p=<0.001)。间隔压迫与患有局部疾病的成年人的生存率改善没有显着相关(HR,0.51;95%CI0.1-2.3;p=0.373)。与成人相比,儿童在诊断后28天内更经常开始治疗(31/33;94%)(41/64;64%,p=0.001)。28天内的治疗与整个队列生存率的提高相关(HR,2.0495%CI,1.1-3.9;p=0.03)。在对患有局部疾病的个体的亚分析中保留了这种关联(HR,5.4;95%CI,1.9-15;p=0.001),只有成年人(HR,5.3,95%CI,1.7-17;p=0.005)。
结论:尤文肉瘤的成人生存率低于儿童,尽管表现相似,肿瘤的特点和治疗。需要进一步研究成人间隔压缩的价值。及时治疗应该是这种疾病的优先事项。
BACKGROUND: Ewing sarcoma is an aggressive malignancy primarily affecting children and adolescents. Limited research is available on treatment practices, clinical course, and survival in adults.
METHODS: A multi-institution retrospective cohort study of all adults (>18 years) and children (≤18 years) with Ewing sarcoma treated in British Columbia, Canada between January 01, 2000 and December 31, 2018.
RESULTS: One-hundred seven individuals (66 adults, 41 children) were included in the analysis. 5-year OS was 58 % in adults and 75 % in children. For individuals with local disease, 5-year OS was 74 % in adults and 84 % in children. Adult status was associated with impaired PFS (HR, 1.8; 95 % CI, 1.0 - 3.1, p=0.04) and OS (HR, 1.8; 95 % CI, 0.9 - 3.5; p=0.088). A Charlson Comorbidity Index (CCI) ≥3 was associated with impaired survival in adults and children (HR, 3.9, 95 % CI, 2.0 - 7.5; p=<0.001); baseline CCIs were not significantly different between groups. Most adults (61/66; 92 %) and all children (41/41; 100 %) received systemic treatment with no significant difference in mean lines of therapy, treatment modalities or agents. Most children received interval-compressed chemotherapy (35/41; 85 %) compared to adults (19/61; 29 %; p=<0.001). Interval-compression was not significantly associated with improved survival in adults with local disease (HR, 0.51; 95 % CI 0.1 - 2.3; p=0.373). Children more often initiated treatment within 28 days of diagnosis (31/33; 94 %) compared to adults (41/64; 64 %, p=0.001). Treatment within 28 days was associated with improved survival in the entire cohort (HR, 2.04 95 % CI, 1.1 - 3.9; p = 0.03). This association was preserved in subanalysis of individuals with local disease (HR, 5.4; 95 % CI, 1.9 - 15; p = 0.001) and only adults (HR, 5.3, 95 % CI, 1.7 - 17; p = 0.005).
CONCLUSIONS: Survival for adults with Ewing sarcoma is inferior to children despite similarities in presentation, tumour characteristics and treatments. Further studies on the value of interval-compression in adults are required. Timely initation of treatment should be a priority for this disease.