Radiotherapy results in decreased time to second cancer in children with Li Fraumeni Syndrome

Emma Woodward; John-Paul Kilday; Stephanie Ng; Anna Kelsey; D Gareth Evans

doi:10.1093/jnci/djaf057

Radiotherapy results in decreased time to second cancer in children with Li Fraumeni Syndrome

Emma Woodward, John-Paul Kilday, Stephanie Ng, Anna Kelsey, D Gareth Evans

Research output: Contribution to journal › Article › peer-review

Abstract

Li Fraumeni syndrome (LFS) arising from germline TP53 mutation results in defective DNA repair and increased risk of multiple primary cancers beginning in childhood. Curative intent radiotherapy is often used to treat childhood cancer, but its impact on children with LFS has not been reviewed.

We undertook a retrospective case-series review of 47 children with a solid cancer diagnosed age less than 16 years to assess time and survival after second cancer diagnosis.

After radiotherapy for the first cancer diagnosis, median time to second primary cancer diagnosis was 13.3 years and median survival 9.7 years. Where no radiotherapy was received, median time to second primary cancer diagnosis was 25.1 years (χ2 = 14.8, P < .0001; Hazard Ratio = 7.9 [95% CI = 2.8 to 22.6]), and median survival of 29.2 years (χ2 = 12.5, P = .004, Hazard Ratio = 3.2 [95% CI = 1.5 to 6.6]).

Radiotherapy for first cancer in children with LFS is associated with adverse outcomes and ought to be considered only in the absence of other potentially curative options. Where unavoidable, second cancer risks must be minimized.

Original language	English
Article number	djaf057
Journal	National Cancer Institute. Journal
Early online date	10 Mar 2025
DOIs	https://doi.org/10.1093/jnci/djaf057
Publication status	Published - 10 Mar 2025

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NIHR Manchester Biomedical Research Centre
Bruce, I. (PI), Lord, G. (CoI), Lennon, R. (CoI), Black, G. (CoI), Wedge, D. (CoI), Morris, A. (CoI), Hussell, T. (CoI), Sharrocks, A. (CoI), Stivaros, S. (CoI), Buch, M. (CoI), Gough, J. (CoI), Kostarelos, K. (CoI), Thistlethwaite, F. (CoI), Kadler, K. (CoI), Barton, A. (CoI), Hyrich, K. (CoI), Mcbeth, J. (CoI), O'Neill, T. (CoI), Vestbo, J. (CoI), Simpson, A. (CoI), Singh, S. (CoI), Smith, J. (CoI), Felton, T. (CoI), Murray, C. (CoI), Griffiths, C. (CoI), Cullum, N. (CoI), Rhodes, L. (CoI), Warren, R. (CoI), Paus, R. (CoI), Dumville, J. (CoI), Viros Usandizaga, A. (CoI), Keavney, B. (CoI), Tomaszewski, M. (CoI), Allan, S. (CoI), Body, R. (CoI), Cartwright, E. (CoI), Heagerty, A. (CoI), Kalra, P. (CoI), Miller, C. (CoI), Rutter, M. (CoI), Smith, C. (CoI), Trafford, A. (CoI), Evans, D. (CoI), Crosbie, E. (CoI), Crosbie, P. (CoI), Harvie, M. (CoI), Howell, S. (CoI), Renehan, A. (CoI), Dive, C. (CoI), Blackhall, F. (CoI), Landers, D. (CoI), Krebs, M. (CoI), Cook, N. (CoI), Clarke, R. (CoI), Taylor, S. (CoI), Jorgensen, C. (CoI), Lorigan, P. (CoI), Jayson, G. (CoI), Valle, J. (CoI), Mccabe, M. (CoI), Armstrong, A. (CoI), Freitas, A. (CoI), Illidge, T. (CoI), Choudhury, A. (CoI), Hoskin, P. (CoI), West, C. (CoI), Van Herk, M. (CoI), Faivre-Finn, C. (CoI), Bristow, R. (CoI), Kirkby, K. (CoI), Birtle, A. (CoI), Mackay, R. (CoI), Radford, J. (CoI), Linton, K. (CoI), Higham, C. (CoI), Munro, K. (CoI), Plack, C. (CoI), Arden Armitage, C. (CoI), Bruce, I. (CoI), Moore, D. (CoI), Saunders, G. (CoI), Stone, M. (CoI), Haddock, G. (CoI), Lewis, S. (CoI), Elliott, R. (CoI), Green, J. (CoI), Lovell, K. (CoI), Morrison, A. (CoI), Shaw, J. (CoI), Bucci, S. (CoI), Ainsworth, J. (CoI), Webb, R. (CoI), Newman, W. (CoI), Banka, S. (CoI), Clayton-Smith, J. (CoI), Payne, K. (CoI), Moldovan, R. (CoI), Wynn, R. (CoI) & Jones, S. (CoI)
1/12/22 → 30/11/27
Project: Research

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@article{ee516027f5a049d3b4a28f3f88f9003b,

title = "Radiotherapy results in decreased time to second cancer in children with Li Fraumeni Syndrome",

abstract = "Li Fraumeni syndrome (LFS) arising from germline TP53 mutation results in defective DNA repair and increased risk of multiple primary cancers beginning in childhood. Curative intent radiotherapy is often used to treat childhood cancer, but its impact on children with LFS has not been reviewed.We undertook a retrospective case-series review of 47 children with a solid cancer diagnosed age less than 16 years to assess time and survival after second cancer diagnosis.After radiotherapy for the first cancer diagnosis, median time to second primary cancer diagnosis was 13.3 years and median survival 9.7 years. Where no radiotherapy was received, median time to second primary cancer diagnosis was 25.1 years (χ2 = 14.8, P < .0001; Hazard Ratio = 7.9 [95\% CI = 2.8 to 22.6]), and median survival of 29.2 years (χ2 = 12.5, P = .004, Hazard Ratio = 3.2 [95\% CI = 1.5 to 6.6]).Radiotherapy for first cancer in children with LFS is associated with adverse outcomes and ought to be considered only in the absence of other potentially curative options. Where unavoidable, second cancer risks must be minimized.",

author = "Emma Woodward and John-Paul Kilday and Stephanie Ng and Anna Kelsey and Evans, \{D Gareth\}",

note = "{\textcopyright} The Author(s) 2025. Published by Oxford University Press.",

year = "2025",

month = mar,

day = "10",

doi = "10.1093/jnci/djaf057",

language = "English",

journal = "National Cancer Institute. Journal",

issn = "0027-8874",

publisher = "Oxford University Press",

}

TY - JOUR

T1 - Radiotherapy results in decreased time to second cancer in children with Li Fraumeni Syndrome

AU - Woodward, Emma

AU - Kilday, John-Paul

AU - Ng, Stephanie

AU - Kelsey, Anna

AU - Evans, D Gareth

PY - 2025/3/10

Y1 - 2025/3/10

N2 - Li Fraumeni syndrome (LFS) arising from germline TP53 mutation results in defective DNA repair and increased risk of multiple primary cancers beginning in childhood. Curative intent radiotherapy is often used to treat childhood cancer, but its impact on children with LFS has not been reviewed.We undertook a retrospective case-series review of 47 children with a solid cancer diagnosed age less than 16 years to assess time and survival after second cancer diagnosis.After radiotherapy for the first cancer diagnosis, median time to second primary cancer diagnosis was 13.3 years and median survival 9.7 years. Where no radiotherapy was received, median time to second primary cancer diagnosis was 25.1 years (χ2 = 14.8, P < .0001; Hazard Ratio = 7.9 [95% CI = 2.8 to 22.6]), and median survival of 29.2 years (χ2 = 12.5, P = .004, Hazard Ratio = 3.2 [95% CI = 1.5 to 6.6]).Radiotherapy for first cancer in children with LFS is associated with adverse outcomes and ought to be considered only in the absence of other potentially curative options. Where unavoidable, second cancer risks must be minimized.

AB - Li Fraumeni syndrome (LFS) arising from germline TP53 mutation results in defective DNA repair and increased risk of multiple primary cancers beginning in childhood. Curative intent radiotherapy is often used to treat childhood cancer, but its impact on children with LFS has not been reviewed.We undertook a retrospective case-series review of 47 children with a solid cancer diagnosed age less than 16 years to assess time and survival after second cancer diagnosis.After radiotherapy for the first cancer diagnosis, median time to second primary cancer diagnosis was 13.3 years and median survival 9.7 years. Where no radiotherapy was received, median time to second primary cancer diagnosis was 25.1 years (χ2 = 14.8, P < .0001; Hazard Ratio = 7.9 [95% CI = 2.8 to 22.6]), and median survival of 29.2 years (χ2 = 12.5, P = .004, Hazard Ratio = 3.2 [95% CI = 1.5 to 6.6]).Radiotherapy for first cancer in children with LFS is associated with adverse outcomes and ought to be considered only in the absence of other potentially curative options. Where unavoidable, second cancer risks must be minimized.

U2 - 10.1093/jnci/djaf057

DO - 10.1093/jnci/djaf057

M3 - Article

C2 - 40059635

SN - 0027-8874

JO - National Cancer Institute. Journal

JF - National Cancer Institute. Journal

M1 - djaf057

ER -

Radiotherapy results in decreased time to second cancer in children with Li Fraumeni Syndrome

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