Risk of soft-tissue sarcoma among 69 460 five-year survivors of childhood cancer in Europe

Research output: Contribution to journalArticle

Authors

  • Chloe J Bright
  • Michael Hawkins
  • David L Winter
  • Daniela Alessi
  • Rodrigue S Allodji
  • Francesca Bagnasco
  • Edit Bárdi
  • Andrea Bautz
  • Julianne Byrne
  • Elizabeth A M Feijen
  • Miranda M Fidler
  • Stanislaw Garwicz
  • Desiree Grabow
  • Thorgerdur Gudmundsdottir
  • Joyeeta Guha
  • Nadia Haddy
  • Momcilo Jankovic
  • Peter Kaatsch
  • Melanie Kaiser
  • Claudia E Kuehni
  • Helena Linge
  • Hilde Øfstaas
  • Cecile M Ronckers
  • Roderick Skinner
  • Jop C Teepen
  • Monica Terenziani
  • Giao Vu-Bezin
  • Finn Wesenberg
  • Thomas Wiebe
  • Carlotta Sacerdote
  • Zsuzsanna Jakab
  • Riccardo Haupt
  • Päivi Lähteenmäki
  • Lorna Zadravec Zaletel
  • Rahel Kuonen
  • Jeanette F Winther
  • Florent de Vathaire
  • Leontien C Kremer
  • Lars Hjorth
  • Raoul Reulen
  • PanCareSurFup Consortium

Colleges, School and Institutes

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.

Details

Original languageEnglish
JournalJournal of the National Cancer Institute
Early online date20 Nov 2017
Publication statusE-pub ahead of print - 20 Nov 2017