Long-term adverse effects of modern Wilms tumour therapies: implications for monitoring

Wilms tumour is the most common kidney tumour in children. Owing to global collaboration and advances in clinical care, 90% of affected children, including those with metastatic disease, can now be cured. Further improvements in this outstanding outcome will depend on implementing strategies to prevent treatment-related mortality and gaining insights into the molecular and clinical drivers of Wilms tumour to introduce tailored therapies. The main treatments for Wilms tumour are nephrectomy and chemotherapy, with radiotherapy used selectively. Wilms tumour therapies can lead to long-term chronic health conditions, such as chronic kidney disease, infertility, second primary neoplasms and cardiovascular disease, despite the use of risk-adapted protocols to optimize the therapeutic index. Research into therapy de-escalation has been enhanced by survivor cohort studies investigating the chronic health conditions associated with specific Wilms tumour therapies. Understanding these relationships and which patients are most susceptible to specific toxic effects is crucial for counselling Wilms tumour survivors and their health-care providers on survivorship care planning. A classification framework could stratify survivors by their risk of treatment-related long-term morbidity, to tailor long-term follow-up monitoring and care.