The rationale for nephron-sparing surgery in unilateral non-syndromic Wilms tumour

Abstract

The central question of nephron-sparing surgery in unilateral non-syndromic Wilms tumour sits at a crossroads between surgery, oncology, and nephrology. There has been a significant paradigm shift in paediatric oncology towards reducing toxicity and addressing long-term treatment-related sequalae amongst childhood cancer survivors. After paediatric nephrectomy and 30-50 years of follow-up, 40% of patients will have chronic kidney disease, including 22% with hypertension and 23% with albuminuria. It is difficult to predict which patients will progress to develop hypertension, reduced glomerular filtration rate, albuminuria, and a higher cardiovascular risk. For these reasons, nephron-sparing surgery when it is technically feasible must be considered. To decrease the incidence of positive surgical margins (viable tumour present at a resection margin), incomplete lymph node sampling, and complications, these procedures should be performed at specialist and experienced reference centres. Based on the impacts of individual treatment pathways, survivors of childhood WT need to be followed through adulthood for early detection of chronic kidney disease, hypertension, and prevention of cardiovascular events.

Keywords: Kidney function; Nephrectomy; Nephroblastoma; Nephron-sparing surgery; Outcome; Wilms tumour.

Fig. 1

Anterior view of a 3D reconstruction illustrating a lower pole Wilms tumour (green) abutting the inferior calyx but well clear of the left renal vein and artery

Fig. 2

Conditions for NSS in unilateral non-syndromic WT from the UMBRELLA protocol (2016) established by the Renal Tumour Study Group of the International Society of Paediatric Oncology. The points marked “*” are discussed further in the manuscript