Risk of Soft-Tissue Sarcoma Among 69 460 Five-Year Survivors of Childhood Cancer in Europe

PanCareSurFup Consortium

Research output: Contribution to journalArticle

Abstract

Background: 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.

Original languageEnglish
Pages (from-to)649-660
Number of pages12
JournalJournal of the National Cancer Institute
Volume110
Issue number6
DOIs
Publication statusPublished - Jun 1 2018

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Sarcoma
Incidence
Confidence Intervals
Leiomyosarcoma
Neoplasms
Retinoblastoma
Neurilemmoma
Survivors
Wilms Tumor
Central Nervous System Neoplasms
Hodgkin Disease
Health Personnel

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Risk of Soft-Tissue Sarcoma Among 69 460 Five-Year Survivors of Childhood Cancer in Europe. / PanCareSurFup Consortium.

In: Journal of the National Cancer Institute, Vol. 110, No. 6, 01.06.2018, p. 649-660.

Research output: Contribution to journalArticle

@article{041bd82a26364a1685038bd434e35a7d,
title = "Risk of Soft-Tissue Sarcoma Among 69 460 Five-Year Survivors of Childhood Cancer in Europe",
abstract = "Background: 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.",
author = "{PanCareSurFup Consortium} and Bright, {Chloe J} and Hawkins, {Mike M} and Winter, {David L} and Daniela Alessi and Allodji, {Rodrigue S} and Francesca Bagnasco and Edit B{\'a}rdi and Andrea Bautz and Julianne Byrne and Feijen, {Elizabeth A M} and Fidler, {Miranda M} and Stanislaw Garwicz and Desiree Grabow and Thorgerdur Gudmundsdottir and Joyeeta Guha and Nadia Haddy and Momcilo Jankovic and Peter Kaatsch and Melanie Kaiser and Kuehni, {Claudia E} and Helena Linge and Hilde {\O}fstaas and Ronckers, {Cecile M} and Roderick Skinner and Teepen, {Jop C} and Monica Terenziani and Giao Vu-Bezin and Finn Wesenberg and Thomas Wiebe and Carlotta Sacerdote and Zsuzsanna Jakab and Riccardo Haupt and P{\"a}ivi L{\"a}hteenm{\"a}ki and Zaletel, {Lorna Zadravec} and Rahel Kuonen and Winther, {Jeanette F} and {de Vathaire}, Florent and Kremer, {Leontien C} and Lars Hjorth and Reulen, {Raoul C}",
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month = "6",
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doi = "10.1093/jnci/djx235",
language = "English",
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TY - JOUR

T1 - Risk of Soft-Tissue Sarcoma Among 69 460 Five-Year Survivors of Childhood Cancer in Europe

AU - PanCareSurFup Consortium

AU - Bright, Chloe J

AU - Hawkins, Mike M

AU - Winter, David L

AU - Alessi, Daniela

AU - Allodji, Rodrigue S

AU - Bagnasco, Francesca

AU - Bárdi, Edit

AU - Bautz, Andrea

AU - Byrne, Julianne

AU - Feijen, Elizabeth A M

AU - Fidler, Miranda M

AU - Garwicz, Stanislaw

AU - Grabow, Desiree

AU - Gudmundsdottir, Thorgerdur

AU - Guha, Joyeeta

AU - Haddy, Nadia

AU - Jankovic, Momcilo

AU - Kaatsch, Peter

AU - Kaiser, Melanie

AU - Kuehni, Claudia E

AU - Linge, Helena

AU - Øfstaas, Hilde

AU - Ronckers, Cecile M

AU - Skinner, Roderick

AU - Teepen, Jop C

AU - Terenziani, Monica

AU - Vu-Bezin, Giao

AU - Wesenberg, Finn

AU - Wiebe, Thomas

AU - Sacerdote, Carlotta

AU - Jakab, Zsuzsanna

AU - Haupt, Riccardo

AU - Lähteenmäki, Päivi

AU - Zaletel, Lorna Zadravec

AU - Kuonen, Rahel

AU - Winther, Jeanette F

AU - de Vathaire, Florent

AU - Kremer, Leontien C

AU - Hjorth, Lars

AU - Reulen, Raoul C

PY - 2018/6/1

Y1 - 2018/6/1

N2 - Background: 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.

AB - Background: 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.

U2 - 10.1093/jnci/djx235

DO - 10.1093/jnci/djx235

M3 - Article

C2 - 29165710

VL - 110

SP - 649

EP - 660

JO - Journal of the National Cancer Institute

JF - Journal of the National Cancer Institute

SN - 0027-8874

IS - 6

ER -