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Management of Wilms tumor: current standard of care

Nature Clinical Practice Urology volume 5, pages 551–560 (2008)Cite this article

Abstract

Wilms tumor is the most common renal malignancy in children. In the 1930s, overall survival for children with Wilms tumor was approximately 30%. Use of multidisciplinary therapy, guided by results from multi-institutional, randomized trials, has substantially improved overall survival to about 90%. Management of Wilms tumor differs substantially between Europe and the US. In Europe, the International Society of Pediatric Oncology protocols call for management of patients with presumptive Wilms tumor with neoadjuvant chemotherapy followed by nephrectomy and further chemotherapy. In the US, protocols developed by the National Wilms Tumor Study Group advise primary nephrectomy followed by a chemotherapy regimen tailored to the pathologic tumor stage. Despite these disparate strategies, overall survival is similar in patients managed according to European and US protocols. Patients with Wilms tumor now have excellent survival. Therefore, current goals aim to reduce the morbidity associated with therapy. Important complications of treatment for Wilms tumor include cardiomyopathy, renal failure, and increased risk of a secondary malignancy. Currently, the role of laparoscopic surgery in management of Wilms tumor remains extremely limited.

Key Points

  • Multidisciplinary therapy has improved overall survival for patients with Wilms tumor to approximately 90%

  • In North America, National Wilms Tumor Study Group protocols dictate primary nephrectomy followed by chemotherapy for most patients who have Wilms tumor

  • In Europe, International Society of Pediatric Oncology protocols call for neoadjuvant chemotherapy in cases of presumptive Wilms tumor; this initial treatment is followed by nephrectomy and further chemotherapy

  • Despite their different strategies, overall survival rates achieved by NWTSG and SIOP approaches are similar

  • Prognostic factors in patients with Wilms tumor include disease stage, histology, patient age, and biological markers; the most powerful prognostic indicator is tumor histology

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Authors and Affiliations

  1. G Sonn is a Senior Urology Resident at Stanford University Medical Center, Stanford, CA.,

    Geoffrey Sonn

  2. LMD Shortliffe is the Stanley McCormick Memorial Professor and Chair of the Department of Urology at Stanford University and Chief of Pediatric Urology at Lucile Salter Packard Children's Hospital, Palo Alto, CA, USA.,

    Linda MD Shortliffe

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Correspondence to Linda MD Shortliffe.

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Sonn, G., Shortliffe, L. Management of Wilms tumor: current standard of care. Nat Rev Urol 5, 551–560 (2008). https://doi.org/10.1038/ncpuro1218

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