Surgery for the complex Wilms tumour

Abstract

The prognosis of children with Wilms tumour has greatly improved since the introduction of adjuvant radiotherapy and chemotherapy more than 70 years ago with a current overall long-term survival approaching 90%. Before this, surgery was the only option with around 20% survival, even in low-risk categories. The focus is now on management options for those patients in specific subgroups, who continue to have lower event-free survival and who suffer from the long-term effects of treatment. These include those with anaplastic histology, bilateral Wilms, biologically high-risk tumours and those that relapse (Aldrink et al. in J Pediatr Surg 54(3):390-397, 2019). The major advances that have already been made in risk assessment and tailoring the chemotherapy/radiotherapy to achieve maximum advantage with minimum toxicity and long-term morbidity have been predicated on safe and complete resection and staging of the tumour. There are, however, still surgical challenges faced with respect to resection of 'complex' tumours; thus, surgeons need to understand their role and advance their expertise in the overall treatment of children with Wilms tumour. These include very large tumours not responding to chemotherapy, ruptured tumours, tumours with intravascular invasion into the inferior vena cava (+/- 10%), heart and hepatic veins (+/- 4%) and Wilms tumours in horseshoe kidneys (< 1%) (Kieran and Ehrlich in Urol Oncol , 2015). This review describes surgical strategies and techniques used in these situations, gleaned from the authors' experience in the surgical management of over 300 children with Wilms tumours in our centres over the last 30 years.