Treatment of Wilms' tumour. Current recommendations

Abstract

Wilms' tumour (nephroblastoma, renal embryoma) is the fifth most common paediatric malignancy, arising from the embryonal tissue of kidneys and first formally described by Max Wilms in his classic 1899 monograph. Until the early part of this century, Wilms' tumour was associated with a less than 20% survival rate. The current survival rate exceeds 80%, primarily due to large multi-institutional trials such as the National Wilms' Tumor Study (NWTS). These studies have refined and defined the roles of surgery, chemotherapy, and radiation in treating Wilms' tumour, based on staging and histology. The dramatic improvement in the prognosis for children with Wilms' tumour, especially over the past 20 years, represents a landmark achievement in the history of paediatric oncology. Specific treatment recommendations are based on the current National Wilms' Tumor Study IV schema. Stages I and II favourable histology patients do not receive radiotherapy, but are treated postoperatively with 'pulsed' or 'conventional' dactinomycin and vincristine; stage III favourable histology requires postoperative abdominal radiotherapy followed by triple agent, 'conventional' or 'pulsed' chemotherapy (dactinomycin, doxorubicin and vincristine). Patients with stage IV favourable histology, stages II to IV anaplastic, clear cell or rhabdoid histology, are treated similarly with aggressive triple-agent chemotherapy, with the addition of radiotherapy to selected sites. Recurrent and adult Wilms' tumours have poor prognoses and are treated with aggressive surgery, radiotherapy and chemotherapy.