Ou Judy Y, Spraker-Perlman Holly, Dietz Andrew C, Smits-Seemann Rochelle R, Kaul Sapna, Kirchhoff Anne C
Cancer Control and Population Sciences, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT 84112, United States; Department of Pediatrics, Division of Pediatric Hematology/Oncology, University of Utah School of Medicine, Salt Lake City, UT 84113, United States.
Department of Pediatrics, Division of Pediatric Hematology/Oncology, University of Utah School of Medicine, Salt Lake City, UT 84113, United States.
Cancer Epidemiol. 2017 Oct;50(Pt A):150-157. doi: 10.1016/j.canep.2017.08.015. Epub 2017 Oct 7.
Survival estimates for soft tissue sarcomas (STS) and malignant bone tumors (BT) diagnosed in pediatric, adolescent, and young adult patients are not easily available. We present survival estimates based on a patient having survived a defined period of time (conditional survival). Conditional survival estimates for the short-term were calculated for patients from diagnosis to the first five years after diagnosis and for patients surviving in the long-term (up to 20 years after diagnosis).
We identified 703 patients who were diagnosed with a STS or BT at age ≤25 years from January 1, 1986 to December 31, 2012 at a large pediatric oncology center in Salt Lake City, Utah, United States. We obtained cancer type, age at diagnosis, primary site, and demographic data from medical records, and vital status through the National Death Index. Cancer stage was available for a subset of the cohort through the Utah Cancer Registry. Cox proportional hazards models, adjusted for age and sex, calculated survival estimates for all analyses.
Short-term survival improves over time for both sarcomas. Short-term survival for STS from diagnosis (Year 0) did not differ by sex, but short-term survival starting from 1-year post diagnosis was significantly worse for male patients (Survival probability 1-year post-diagnosis [SP1]:77% [95% CI:71-83]) than female patients (SP1:86% [81-92]). Survival for patients who were diagnosed at age ≤10 years (Survival probability at diagnosis [SP0]:85% [79-91]) compared to diagnosis at ages 16-25 years (SP0:67% [59-75]) was significantly better at all time-points from diagnosis to 5-years post-diagnosis. Survival for axial sites (SP0:69% [63-75]) compared to extremities (SP0:84% [79-90]) was significantly worse from diagnosis to 1-year post-diagnosis. Survival for axial BT (SP0: 64% [54-74] was significantly worse than BT in the extremities (SP0:73% [68-79]) from diagnosis to 3-years post diagnosis. Relapsed patients of both sarcoma types had significantly worse short-term survival than non-relapsed patients. Long-term survival for STS in this cohort is 65% at diagnosis, and improves to 86% 5-years post-diagnosis. BT survival improves from 51% at diagnosis to 78% at 5-years post-diagnosis.
Conditional survival for short- and long-term STS and BT improve as time from diagnosis increases. Short-term survival was significantly affected by patients' sex, age at diagnosis, cancer site, and relapse status.
关于小儿、青少年及年轻成人患者诊断的软组织肉瘤（STS）和恶性骨肿瘤（BT）的生存估计不易获取。我们给出基于患者存活特定时间段（条件生存）的生存估计。计算了从诊断到诊断后前五年的短期条件生存估计以及长期（诊断后长达20年）存活患者的条件生存估计。
我们确定了1986年1月1日至2012年12月31日在美国犹他州盐湖城一家大型儿科肿瘤中心诊断为年龄≤25岁的STS或BT的703例患者。我们从病历中获取癌症类型、诊断时年龄、原发部位和人口统计学数据，并通过国家死亡指数获取生命状态。通过犹他癌症登记处可获得该队列一部分患者的癌症分期。针对年龄和性别进行调整的Cox比例风险模型计算了所有分析的生存估计。
两种肉瘤的短期生存均随时间改善。STS从诊断时（第0年）起的短期生存无性别差异，但诊断后1年起男性患者的短期生存明显差于女性患者（诊断后1年生存概率[SP1]：77%[95%CI：71 - 83]）（SP1：86%[81 - 92]）。诊断时年龄≤10岁的患者（诊断时生存概率[SP0]：85%[79 - 91]）与16 - 25岁诊断的患者（SP0：67%[59 - 75]）相比，从诊断到诊断后5年的所有时间点生存均明显更好。从诊断到诊断后1年，轴向部位的生存（SP0：69%[63 - 75]）与四肢部位相比（SP0：84%[79 - 90]）明显更差。从诊断到诊断后3年，轴向BT的生存（SP0：64%[54 - 74]）明显差于四肢部位的BT（SP0：73%[68 - 79]）。两种肉瘤类型的复发患者短期生存均明显差于未复发患者。该队列中STS的长期生存诊断时为65%，诊断后5年提高到86%。BT生存从诊断时的51%提高到诊断后5年的78%。
STS和BT的短期和长期条件生存随诊断后时间增加而改善。短期生存受患者性别、诊断时年龄、癌症部位和复发状态的显著影响。
