Saga of Wilms' tumor: Lessons learnt from the past

Wilms′ tumor (WT) represents 6% of childhood cancers. Recent advances in molecular biology have significant implications for the clinical management. A dramatic improvement in overall cure has resulted from the adoption of multimodality treatment during the past few decades. National Wilms′ Tumor Study (NWTS), and the Sociιtι Internationale d′Oncologie Pιdiatrique (SIOP) have laid down the guidelines for standardized treatment of WT, though differing in the diagnostic and therapeutic approach. Both these groups currently aim at intensifying treatment for patients with poor prognosticators while appropriating the therapy to reduce long-term complications for those with favorable prognostic features. Challenges faced in developing nations include poverty, malnutrition, ignorance, and presentation in advanced stages coupled with limited facilities that are necessary for total management of these cases. In this article, we have discussed our approach to deal with patients with nephroblastoma, reviewed the literature on the current management strategies and the long-term outcome. Most countries have adopted the NWTS protocols, while others especially in Europe, South America and some Asian countries follow the SIOP regimen. Both have their advantages and weaknesses and maynot necessarily be suitable to the setup in developing countries. We have discussed the controversial issues in the management of WT including the timing of biopsy, type of biopsy, investigative approach, role of chemotherapy / radiotherapy, management of bilateral Wilms′ tumor and parenchymal sparing renal surgery. Despite deviating from NWTS at various points, the overall results have remained satisfactory. Thus, developing countries might adopt their own protocols depending on the prevalent situations and facilities available to them to treat such patients.