Marina Neyssa M, Liu Qi, Donaldson Sarah S, Sklar Charles A, Armstrong Gregory T, Oeffinger Kevin C, Leisenring Wendy M, Ginsberg Jill P, Henderson Tara O, Neglia Joseph P, Stovall Marilyn A, Yasui Yutaka, Randall R Lor, Geller David S, Robison Leslie L, Ness Kirsten K
Department of Pediatrics, Stanford University and Lucile Packard Children's Hospital, Palo Alto, California.
School of Public Health, University of Alberta, Edmonton, Alberta, Canada.
Cancer. 2017 Jul 1;123(13):2551-2560. doi: 10.1002/cncr.30627. Epub 2017 Feb 21.
Ewing sarcoma survivors (ESSs) are at increased risk for treatment-related complications. The incidence of treatment-related morbidity and late mortality with aging is unknown.
This study reports survival probabilities, estimated with the Kaplan-Meier method, and the cumulative incidence of cause-specific mortality and chronic conditions among ESSs in the Childhood Cancer Survivor Study who were treated between 1970 and 1986. Piecewise exponential models were used to estimate relative rates (RRs) and 95% confidence intervals (CIs) for these outcomes. Chronic conditions were graded with the Common Terminology Criteria for Adverse Events (version 4.03).
Among 404 5-year ESSs (median age at last follow-up, 34.8 years; range, 9.1-54.8 years), the 35-year survival rate was 70% (95% CI, 66%-74%). Late recurrence (cumulative incidence at 35 years, 15.1%) was the most common cause of death, and it was followed by treatment-related causes (11.2%). There were 53 patients with subsequent neoplasms (SNs; cumulative incidence at 35 years, 24.0%), and 38 were malignant (14.3% at 35 years). The standardized incidence ratios were 377.1 (95% CI, 172.1-715.9) for osteosarcoma, 28.9 (95% CI, 3.2-104.2) for acute myeloid leukemia, 14.9 (95% CI, 7.9-25.5) for breast cancer, and 13.1 (95% CI, 4.8-28.5) for thyroid cancer. Rates of chronic conditions were highest for musculoskeletal (RR, 18.1; 95% CI, 12.8-25.7) and cardiac complications (RR, 1.8; 95% CI, 1.4-2.3). Thirty-five years after the diagnosis, the cumulative incidences of any chronic conditions and 2 or more chronic conditions were 84.6% (95% CI, 80.4%-88.8%) and 73.8% (95% CI, 67.8%-79.9%), respectively.
With extended follow-up, ESSs' risk for late mortality and SNs does not plateau. Treatment-related chronic conditions develop years after therapy, and this supports the need for lifelong follow-up. Cancer 2017;123:2551-60. © 2017 American Cancer Society.
尤因肉瘤幸存者（ESSs）发生治疗相关并发症的风险增加。与治疗相关的发病率及随年龄增长的晚期死亡率尚不清楚。
本研究报告了1970年至1986年间接受治疗的儿童癌症幸存者研究中ESSs的生存概率（采用Kaplan-Meier法估计）以及特定病因死亡率和慢性病的累积发病率。采用分段指数模型估计这些结局的相对率（RRs）和95%置信区间（CIs）。根据不良事件通用术语标准（第4.03版）对慢性病进行分级。
在404例5年ESSs患者中（末次随访时的中位年龄为34.8岁；范围为9.1 - 54.8岁），35年生存率为70%（95%CI，66% - 74%）。晚期复发（35年累积发病率为15.1%）是最常见的死亡原因，其次是治疗相关原因（11.2%）。有53例患者发生后续肿瘤（SNs；35年累积发病率为24.0%），其中38例为恶性肿瘤（35年时为14.3%）。骨肉瘤的标准化发病比为377.1（95%CI，172.1 - 715.9），急性髓系白血病为28.9（95%CI，3.2 - 104.2），乳腺癌为14.9（95%CI，7.9 - 25.5），甲状腺癌为13.1（95%CI，4.8 - 28.5）。肌肉骨骼疾病（RR，18.1；95%CI，12.8 - 25.7）和心脏并发症（RR，1.8；95%CI，1.4 - 2.3）的慢性病发生率最高。诊断后35年，任何慢性病和两种或更多慢性病的累积发病率分别为84.6%（95%CI，80.4% - 88.8%）和73.8%（95%CI，67.8% - 79.9%）。
随着随访时间延长，ESSs的晚期死亡率和SNs风险并未趋于平稳。治疗相关慢性病在治疗数年后出现，这支持了终身随访的必要性。《癌症》2017年；123：2551 - 60。©2017美国癌症协会。
