Author/Editor		Bright, Chloe J.; Hawkins, Mike M.; Winter, David L.; Alessi, Daniela; Allodji, Rodrigue S.; Bagnasco, Francesca; Bárdi, Edit; Bautz, Andrea; Byrne, Julianne; Zadravec-Zaletel, Lorna
Title		Risk of soft-tissue sarcoma among 69 460 five-year survivors of childhood cancer in Europe
Type		članek
Vol. and No.		Letnik 110, št. 6
Publication year		2018
Volume		str. 1-12
ISSN		0027-8874 - Journal of the National Cancer Institute
Language		eng
Abstract		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. Methods 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. Results 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. Conclusions 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.
Keywords		rak v otroštvu pozne posledice sarkom sarkom mehkih tkiv childhood cancer late effects soft-tissue sarcoma