Renal tumours in Australian children: 30 years of incidence, outcome and second primary malignancy data from the Australian Childhood Cancer Registry

Brendan C. Jones, Danny R. Youlden, Thomas P. Cundy, Michael E. O'Callaghan, Jonathan Karpelowsky, Joanne F. Aitken, Craig A. McBride

    Research output: Contribution to journalArticlepeer-review

    Abstract

    Aim: This paper describes the incidence and outcomes of childhood renal malignancies in Australia using national population-based data from the Australian Childhood Cancer Registry. Methods: De-identified data for children (0–14 years) diagnosed with renal malignancies from 1983 to 2015 inclusive were extracted. Cause-specific (CSS) and event-free survival up to 20 years from diagnosis were estimated using the cohort method. Adjusted excess mortality hazard ratios were calculated using a multivariable flexible parametric survival model. Details relating to second primary malignancies (SPMs) were also examined. Results: There were 1046 children diagnosed with renal malignancies in Australia between 1983 and 2015 (91% nephroblastoma), generating an annual age-standardised incidence rate of 8 per million children, which remained constant over the study period. CSS was 89% (95% confidence interval = 87–91%) and 88% (86–90%) at 5 and 20 years, respectively, and 5-year event-free survival was 82% (80–84%). Five-year CSS did not change over the study period and was highest for nephroblastoma (91%). Of the 94% of patients achieving remission, 15% relapsed and subsequent 5-year CSS was 49% (40%–58%). Eleven children were diagnosed with SPM (standardised incidence ratio = 2.9, 95% confidence interval = 1.6–5.3, P < 0.001), and five of them (45%) died within 5 years of the second diagnosis. Conclusions: Children treated for renal malignancies in Australia have excellent long-term survival, which is unchanged since 1983. SPMs are uncommon following treatment for childhood renal cancer but carry a poor prognosis. Relapse carries a similarly poor prognosis to SPM but is more common. These data are comparable to registry outcomes in similarly developed nations.

    Original languageEnglish
    Pages (from-to)908-916
    Number of pages9
    JournalJournal of Paediatrics and Child Health
    Volume56
    Issue number6
    DOIs
    Publication statusPublished - Jun 2020

    Keywords

    • kidney neoplasm
    • neoplasms, second primary
    • paediatrics
    • recurrence
    • registries

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