Abstract

Treatment results of Wilms tumors have been impressively improved over the past 50 years resulting in a stage-independent overall survival of greater than 90%. However, unsatisfying treatment results still remain in children with high-risk tumors and tumor relapses. This review highlights the current concepts of Wilms tumor surgery as a cornerstone of the treatment strategy for this malignancy. A selective literature review focusing on the past 5 years served as the basis for this article. Nephron-sparing surgery is associated with an analogue outcome compared to tumor nephrectomy in unilateral Wilms tumors. The surgical panel of the International Society of Pediatric Oncology (SIOP) group has recently introduced a novel nomenclature for organ-preserving resection procedures in order to facilitate a prospective comparison of data. The minimally invasive approach represents an alternative technique with adequate outcome. In bilateral disease, nephron-sparing procedures are gold standard. Complete resection of lung and liver metastases has a significant impact on patients' survival. Surgical guidelines for nephron-sparing surgery and minimally invasive tumor nephrectomy need to be established and implemented within newly formulated treatment protocols of the different national and international treatment trials. Risk stratification of patients needs to be more individualized with the aim of reducing late effects while at least maintaining the same survival rates. The unsatisfying treatment results of tumor relapses-associated with low patient numbers within the different trials-emphasize the need for international collaboration.

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