Survival for Children Diagnosed With Wilms Tumour (2012-2022) Registered in the UK and Ireland Improving Population Outcomes for Renal Tumours of Childhood (IMPORT) Study.
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BACKGROUND: The Improving Population Outcomes for Renal Tumours of childhood (IMPORT) is a prospective clinical observational study capturing detailed demographic and outcome data on children and young people diagnosed with renal tumours in the United Kingdom and the Republic of Ireland. METHODS: Data were extracted from the IMPORT database for all newly diagnosed cases of Wilms tumour (WT) registered between November 2012 and December 2022. Kaplan-Meier survival was estimated at one, three and five years. For children aged 0-14 years with unilateral WT, Cox proportional hazards models were used to examine patient/tumour characteristics in relation to the excess hazard of death. RESULTS: Among 620 patients, 570 had unilateral and 50 (8%) bilateral disease; 582 (94%) patients received pre-operative chemotherapy and 38 (6%) immediate surgery. Median age was 41 months (IQR: 41-65). Median tumour volume at diagnosis was 595 cm3 (IQR: 348-914) and 228 cm3 (IQR: 83-535) after pre-operative chemotherapy, with 123/443 (28%) ≥500 cm3. Of 570 patients with unilateral WT, 419 (73.5%) had localised and 151 (26.5%) metastatic disease. Five-year survival was highest for children aged 0-4 years (94.8%). Five-year survival by Toronto stage (assessed at nephrectomy) was 97.0% (stage I/yI), 93.8% (stage II/yII), 85.2% (stage III/yIII) and 88.9% (stage IV), and by histology 74.6% (high risk), 95.2% (intermediate risk) and 100% (low risk). In univariate analysis, histological risk group, age (</≥ 4 years), stage and tumour volume ≥500 cm3 after pre-operative chemotherapy were significantly associated with excess risk of death. In multivariate modelling, only high-risk histology (HR 10.8, 95% CI: 4.0-29.1) retained significance. CONCLUSIONS: Five-year survival for children with WT in the United Kingdom and Ireland is generally above 90%, with high-risk histology as the most important adverse prognostic factor. However, median tumour volume at diagnosis and after pre-operative chemotherapy remains large.