Wilms Tumor



Wilms tumor (WT), or nephroblastoma, is the most common primary malignant renal tumor of childhood. It is the second most common solid organ abdominal tumor encountered in childhood, accounting for 6% of all pediatric tumors. The annual incidence is 8.1 per million children. This results in 600–700 new cases each year in North America. Outcomes for children with WT have improved dramatically over the last 50 years, with long-term survival in both North America and European trials approaching 85%. Moreover, many of the low-stage tumors have survival rates between 95 and 99%. The treatment strategy for children with WT has also evolved. It is currently based on traditional risk factors, such as stage and histology, as well as genetic markers, response to therapy and consideration of the risk of late effects. The goal of “risk-based management” is to maintain excellent outcomes but to spare children with low-risk tumors the long-term side effects of intensive chemo- and radiation therapy and to use more intense therapy for high-risk tumors to minimize recurrence rates (Table 92.1). Risk-based therapy requires a multidisciplinary team that includes oncologists, radiologists, surgeons, radiation oncologists, pathologists, social workers and nurses. Surgeons play a critical role in diagnosis, staging and treatment, and their technical skill and judgment have a direct impact on therapeutic decision-making and patient outcome. The current outcome data for children with WT from the National Wilms Tumor Study are shown in Table 92.2.


Inferior Vena Cava Renal Vein Partial Nephrectomy Tumor Thrombus Contralateral Kidney 
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Suggested Reading

  1. Dome JS, Cotton CA, Perlman EJ, et al. Treatment of anaplastic ­histology Wilms’ tumor: results from the fifth National Wilms’ Tumor Study. J Clin Oncol. 2006;24(15):2352–8.CrossRefPubMedGoogle Scholar
  2. Ehrlich PF, Ritchey ML, Hamilton TE, et al. Quality assessment for Wilms’ tumor: a report from the National Wilms’ Tumor Study-5. J Pediatr Surg. 2005;40(1):208–12.CrossRefPubMedGoogle Scholar
  3. Ehrlich PF, Hamilton TE, Grundy PE, et al. The value of surgery in directing therapy of Wilms tumor patients with pulmonary disease: a report from The National Wilms Tumor Study Group (NWTS -5). J Pediatr Surg. 2006;41(1):162–7.CrossRefPubMedGoogle Scholar
  4. Grundy PE, Breslow N, Li S, et al. Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable-­histology Wilms tumor: a report from the National Wilms Tumor Study Group. J Clin Oncol. 2005;23(29):7312–21.CrossRefPubMedGoogle Scholar
  5. Hamilton TE, Green DM, Perlman EJ, et al. Bilateral Wilms’ tumor with anaplasia: lessons from the National Wilms’ Tumor Study. J Pediatr Surg. 2006;41(10):1641–4.CrossRefPubMedGoogle Scholar
  6. Perlman EJ, Faria P, Soares A, Hoffer FA, et al. Hyperplastic perilobar nephroblastomatosis: Long term survival in 52 patients. Pediatr Blood Cancer. 2006;46:203–21.CrossRefPubMedGoogle Scholar
  7. Ritchey ML. Renal sparing surgery for Wilms tumor. J Urol. 2005;174(4 Pt 1):1172–3.CrossRefPubMedGoogle Scholar
  8. Shamberger RC, Guthrie KA, Ritchey ML, et al. Surgery related factors and local reccurance of Wilms tumor in the National Wilms tumor study 4. Ann Surg. 1999;229(2):292–7.CrossRefPubMedGoogle Scholar
  9. Shamberger RC, Ritchey ML, Haase GM, et al. Intravascular extension of Wilms tumor. Ann Surg. 2001;234(1):116–21.CrossRefPubMedGoogle Scholar
  10. Shamberger RC, Haase G, Argani P. Bilateral Wilms’ tumors with progressive or nonresponsive disease. J Pediatr Surg. 2006;41(4):652–7.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of Pediatric SurgeryUniversity of Michigan, CS Mott Children’s HospitalAnn ArborUSA

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