Center for Childhood Cancer Survivor Studies, Institute of Applied Health Research, Robert Aitken Building, University of Birmingham, Birmingham, UK.
Childhood Cancer Registry of Piedmont, Cancer Epidemiology Unit, Department of Medical Sciences, University of Turin and AOU Città della Salute e della Scienza di Torino, Torino, Italy.
J Natl Cancer Inst. 2018 Jun 1;110(6):649-660. doi: 10.1093/jnci/djx235.
Childhood cancer survivors are at risk of subsequent primary soft-tissue sarcomas (STS), but the risks of specific STS histological subtypes are unknown. We quantified the risk of STS histological subtypes after specific types of childhood cancer.
We pooled data from 13 European cohorts, yielding a cohort of 69 460 five-year survivors of childhood cancer. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated.
Overall, 301 STS developed compared with 19 expected (SIR = 15.7, 95% confidence interval [CI] = 14.0 to 17.6). The highest standardized incidence ratios were for malignant peripheral nerve sheath tumors (MPNST; SIR = 40.6, 95% CI = 29.6 to 54.3), leiomyosarcomas (SIR = 29.9, 95% CI = 23.7 to 37.2), and fibromatous neoplasms (SIR = 12.3, 95% CI = 9.3 to 16.0). SIRs for MPNST were highest following central nervous system tumors (SIR = 80.5, 95% CI = 48.4 to 125.7), Hodgkin lymphoma (SIR = 81.3, 95% CI = 35.1 to 160.1), and Wilms tumor (SIR = 76.0, 95% CI = 27.9 to 165.4). Standardized incidence ratios for leiomyosarcoma were highest following retinoblastoma (SIR = 342.9, 95% CI = 245.0 to 466.9) and Wilms tumor (SIR = 74.2, 95% CI = 37.1 to 132.8). AERs for all STS subtypes were generally low at all years from diagnosis (AER < 1 per 10 000 person-years), except for leiomyosarcoma following retinoblastoma, for which the AER reached 52.7 (95% CI = 20.0 to 85.5) per 10 000 person-years among patients who had survived at least 45 years from diagnosis of retinoblastoma.
For the first time, we provide risk estimates of specific STS subtypes following childhood cancers and give evidence that risks of MPNSTs, leiomyosarcomas, and fibromatous neoplasms are particularly increased. While the multiplicative excess risks relative to the general population are substantial, the absolute excess risk of developing any STS subtype is low, except for leiomyosarcoma after retinoblastoma. These results are likely to be informative for both survivors and health care providers.
儿童癌症幸存者存在发生后续原发性软组织肉瘤（STS）的风险，但特定 STS 组织学亚型的风险尚不清楚。我们定量评估了特定类型儿童癌症后 STS 组织学亚型的风险。
我们汇总了来自 13 个欧洲队列的数据，得出了 69460 名 5 年儿童癌症幸存者的队列。计算了标准化发病比（SIR）和绝对超额风险（AER）。
总体而言，有 301 例 STS 发展，预期为 19 例（SIR=15.7，95%置信区间[CI]为 14.0 至 17.6）。标准化发病比最高的是恶性外周神经鞘瘤（MPNST；SIR=40.6，95%CI=29.6 至 54.3）、平滑肌肉瘤（SIR=29.9，95%CI=23.7 至 37.2）和纤维瘤性肿瘤（SIR=12.3，95%CI=9.3 至 16.0）。中枢神经系统肿瘤（SIR=80.5，95%CI=48.4 至 125.7）、霍奇金淋巴瘤（SIR=81.3，95%CI=35.1 至 160.1）和肾母细胞瘤（SIR=76.0，95%CI=27.9 至 165.4）后 MPNST 的 SIR 最高。平滑肌肉瘤的 SIR 最高的是视网膜母细胞瘤（SIR=342.9，95%CI=245.0 至 466.9）和肾母细胞瘤（SIR=74.2，95%CI=37.1 至 132.8）。所有 STS 亚型的 AER 在诊断后所有年份通常都较低（AER<1/10000 人年），但视网膜母细胞瘤后平滑肌肉瘤除外，在至少有 45 年视网膜母细胞瘤生存的患者中，AER 达到 52.7（95%CI=20.0 至 85.5）/10000 人年。
我们首次提供了儿童癌症后特定 STS 亚型的风险估计，并提供了证据表明 MPNST、平滑肌肉瘤和纤维瘤性肿瘤的风险特别增加。虽然与一般人群相比，倍增超额风险很大，但发生任何 STS 亚型的绝对超额风险较低，除了视网膜母细胞瘤后平滑肌肉瘤。这些结果可能对幸存者和医疗保健提供者都有帮助。
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