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Current Management of Wilms’ Tumor

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Abstract

Wilms’ tumor is the most common malignant renal tumor in children. Survival has improved dramatically over time as a result of prospective randomized clinical trials conducted by the pediatric cooperative cancer groups. Current research is directed toward identifying low-risk patients for whom a reduction in treatment intensity would decrease long-term morbidity. This article reviews the most recent advances in the biology and treatment of children with Wilms’ tumor.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Ruteshouser EC, Huff V: Familial Wilms tumor. Am J Med Genet C Semin Med Genet 2004, 129C:29–34.

    Article  PubMed  Google Scholar 

  2. Ruteshouser EC, Robinson SM, Huff V: Wilms tumor genetics: mutations in WT1, WTX, and CTNNB1 account for only about one-third of tumors. Genes Chromosomes Cancer 2008, 47:461–470.

    Article  CAS  PubMed  Google Scholar 

  3. Coppes MJ, Huff V, Pelletier J: Denys-Drash syndrome: relating a clinical disorder to genetic alterations in the tumor suppressor gene WT1. J Pediatr 1993, 123:673–678.

    Article  CAS  PubMed  Google Scholar 

  4. Little SE, Hanks SP, King-Underwood L, et al.: Frequency and heritability of WT1 mutations in nonsyndromic Wilms’ tumor patients: a UK Children’s Cancer Study Group Study. J Clin Oncol 2004, 22:4140–4106.

    Article  CAS  PubMed  Google Scholar 

  5. Breslow NE, Norris R, Norkool PA, et al.: Characteristics and outcomes of children with the Wilms tumor-Aniridia syndrome: a report from the National Wilms Tumor Study Group. J Clin Oncol 2003, 21:4579–4585.

    Article  PubMed  Google Scholar 

  6. Breslow NE, Takashima JR, Ritchey ML, et al.: Renal failure in the Denys-Drash and Wilms’ tumor-aniridia syndromes. Cancer Res 2000, 60:4030–4032.

    CAS  PubMed  Google Scholar 

  7. Morrison AA, Viney RL, Saleem MA, Ladomery MR: New insights into the function of the Wilms tumor suppressor gene in podocytes. Am J Physiol Renal Physiol 2008, 295:F12–F17.

    Article  CAS  PubMed  Google Scholar 

  8. Dahan K, Kamal M, Noel LH, et al.: Small glomeruli in WAGR (Wilms Tumor, Aniridia, Genitourinary Anomalies and Mental Retardation) syndrome. Am J Kidney Dis 2007, 49:793–800.

    Article  PubMed  Google Scholar 

  9. van Heyningen V, Hoovers JM, de Kraker J, et al.: Raised risk of Wilms tumour in patients with aniridia and submicroscopic WT1 deletion. J Med Genet 2007, 44:787–790.

    Article  PubMed  Google Scholar 

  10. Porteus MH, Narkool P, Neuberg D, et al.: Characteristics and outcome of children with Beckwith-Wiedemann syndrome and Wilms' tumor: a report from the National Wilms Tumor Study Group. J Clin Oncol 2000, 18:2026–2031.

    CAS  PubMed  Google Scholar 

  11. DeBaun MR, Siegel MJ, Choyke PL: Nephromegaly in infancy and early childhood: a risk factor for Wilms tumor in Beckwith-Wiedemann syndrome. J Pediatr 1998, 132:401–404.

    Article  CAS  PubMed  Google Scholar 

  12. Ravenel JD, Broman KW, Perlman EJ, et al.: Loss of imprinting of insulin-like growth factor-II (IGF2) gene in distinguishing specific biologic subtypes of Wilms tumor. J Natl Cancer Inst 2001, 93:1698–1703.

    Article  CAS  PubMed  Google Scholar 

  13. Rivera MN, Kim WJ, Wells J, et al.: An X chromosome gene, WTX, is commonly inactivated in Wilms tumor. Science 2007, 315:642–645.

    Article  CAS  PubMed  Google Scholar 

  14. Wittmann S, Zirn B, Alkassar M, et al.: Loss of 11q and 16q in Wilms tumors is associated with anaplasia, tumor recurrence, and poor prognosis. Genes Chromosomes Cancer 2007, 46:163–170.

    Article  CAS  PubMed  Google Scholar 

  15. Grundy PE, Breslow NE, 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, 23:7312–7321.

    Article  CAS  PubMed  Google Scholar 

  16. Messahel B, Williams R, Ridolfi A, et al.: Allele loss at 16q defines poorer prognosis Wilms tumour irrespective of treatment approach in the UKW1-3 clinical trials: a Children’s Cancer and Leukaemia Group (CCLG) Study. Eur J Cancer 2009, 45:819–826.

    Article  CAS  PubMed  Google Scholar 

  17. Dome JS, Bockhold CA, Li SM, et al.: High telomerase RNA expression level is an adverse prognostic factor for favorable-histology Wilms’ tumor. J Clin Oncol 2005, 23:9138–9145.

    Article  CAS  PubMed  Google Scholar 

  18. Maciel EO, Carvalhal GF, da Silva VD, et al.: Increased tissue factor expression and poor nephroblastoma prognosis. J Urol 2009, 182:1594–1599.

    Article  PubMed  Google Scholar 

  19. Huang CC, Gadd S, Breslow N, et al.: Predicting relapse in favorable histology Wilms tumor using gene expression analysis: a report from the Renal Tumor Committee of the Children’s Oncology Group. Clin Cancer Res 2009, 15:1770–1778.

    Article  CAS  PubMed  Google Scholar 

  20. •• Scott RH, Walker L, Olsen OE, et al.: Surveillance for Wilms tumour in at-risk children: pragmatic recommendations for best practice. Arch Dis Child 2006, 91:995–999. This is an excellent review of the role of screening for WT in at-risk children. This article provides evidence-based recommendations for surveillance of these children.

    Article  CAS  PubMed  Google Scholar 

  21. Choyke PL, Siegel MJ, Craft AW, et al.: Screening for Wilms tumor in children with Beckwith-Wiedemann syndrome or idiopathic hemihypertrophy. Med Pediatr Oncol 1999, 32:196–200.

    Article  CAS  PubMed  Google Scholar 

  22. Coppes MJ, Arnold M, Beckwith JB, et al.: Factors affecting the risk of contralateral Wilms tumor development: a report from the National Wilms Tumor Study Group. Cancer 1999, 85:1616–1625.

    Article  CAS  PubMed  Google Scholar 

  23. Perlman E: Pediatric Renal Tumors: Practical Updates for the Pathologist. Pediatr Dev Pathol 2005, 8:320–338.

    Article  PubMed  Google Scholar 

  24. 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, 24:2352–2358.

    Article  PubMed  Google Scholar 

  25. •• Perlman EJ, Faria P, Soares A, et al.: Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients. Pediatr Blood Cancer 2006, 46:203–221. This is the first large series of children with diffuse hyperplastic nephrogenic rests or nephroblastomatosis. This article provides some evidence for more aggressive management of these patients to include routine chemotherapy.

    Article  PubMed  Google Scholar 

  26. Hamilton TE, Green DM, Perlman EJ, et al.: Bilateral Wilms’ tumor with anaplasia: lessons from the National Wilms’ Tumor Study. J Pediatr Surg 2006, 41:1641–1644.

    Article  PubMed  Google Scholar 

  27. Weirich A, Leuschner I, Harms D, et al.: Clinical impact of histologic subtypes in localized non-anaplastic nephroblastoma treated according to the trial and study SIOP-9/GPOH. Ann Oncol 2001, 12:311–319.

    Article  CAS  PubMed  Google Scholar 

  28. Boccon-Gibod, L, Rey A, Sandstedt B, et al.: Complete necrosis induced by preoperative chemotherapy in Wilms tumor as an indicator of low risk: report of the International Society of Paediatric Oncology (SIOP) nephroblastoma trial and study 9. Med Pediatr Oncol 2000, 34:183–190.

    Article  CAS  PubMed  Google Scholar 

  29. Reinhard HO, Semler D, Burger, et al.: Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms tumor. Klin Padiatr 2004, 216:132–140.

  30. Beckwith JB: Nephrogenic rests and the pathogenesis of Wilms tumor: developmental and clinical considerations. Am J Med Genet 1998, 79:268–273.

    Article  CAS  PubMed  Google Scholar 

  31. Fukuzawa R, Anaka MR, Heathcott RW, et al.: Wilms tumour histology is determined by distinct types of precursor lesions and not epigenetic changes. J Pathol 2008, 215:377–387.

    Article  CAS  PubMed  Google Scholar 

  32. Ritchey M, Daley S, Shamberger RC, et al.: Ureteral extension in Wilms’ tumor: a report from the National Wilms’ Tumor Study Group (NWTSG). J Pediatr Surg 2008, 43:1625–1629.

    Article  PubMed  Google Scholar 

  33. Maas MH, Cransberg K, van Grotel M, et al.: Renin-induced hypertension in Wilms tumor patients. Pediatr Blood Cancer 2007, 48:500–503.

    Article  CAS  PubMed  Google Scholar 

  34. Vujanic GM, Kelsey A, Mitchell C, et al.: The role of biopsy in the diagnosis of renal tumors of childhood: results of the UKCCSG Wilms tumor study 3. Med Pediatr Oncol 2003, 40:18–22.

    Article  PubMed  Google Scholar 

  35. Tournade MF, Com-Nougue C, de Kraker J, et al.: Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms’ tumor in children older than 6 months: results of the Ninth International Society of Pediatric Oncology Wilms’ Tumor Trial and Study. J Clin Oncol 2001, 19:488–500.

    CAS  PubMed  Google Scholar 

  36. Shamberger RC, Ritchey ML, Haase GM, et al.: Intravascular extension of Wilms tumor. Ann Surg 2001, 234:116–121.

    Article  CAS  PubMed  Google Scholar 

  37. Schenk JP, Graf N, Gunther P, et al.: Role of MRI in the management of patients with nephroblastoma. Eur Radiol 2008, 18:683–691.

    Article  PubMed  Google Scholar 

  38. Ritchey ML, Shamberger RC, Hamilton T, et al.: Fate of bilateral renal lesions missed on preoperative imaging: a report from the National Wilms Tumor Study Group. J Urol 2005, 174:1519–1521; discussion 1521.

    Google Scholar 

  39. Shamberger RC, Guthrie KA, Ritchey ML, et al.: Surgery-related factors and local recurrence of Wilms tumor in National Wilms Tumor Study 4. Ann Surg 1999, 229:292–297.

    Article  CAS  PubMed  Google Scholar 

  40. Kalapurakal JA, Li SM, Breslow NE, et al.: Intraoperative spillage of favorable histology Wilms tumor cells: influence of irradiation and chemotherapy regimens on abdominal recurrence. A report from the National Wilms Tumor Study Group. Int J Radiat Oncol Biol Phys 2009 (in press).

  41. Duarte RJ, Denes FT, Cristofani LM, et al.: Laparoscopic nephrectomy for Wilms’ tumor. Expert Rev Anticancer Ther 2009, 9:753–761.

    Article  PubMed  Google Scholar 

  42. Godzinski J, Tournade MF, de Kraker J, et al.: Rarity of surgical complications after postchemotherapy nephrectomy for nephroblastoma. Experience of the International Society of Paediatric Oncology-Trial and Study “SIOP-9.” International Society of Paediatric Oncology Nephroblastoma Trial and Study Committee. Eur J Pediatr Surg 1998, 8:83–86.

    Article  CAS  PubMed  Google Scholar 

  43. Barber TD, Wickiser JE, Wilcox DT, et al.: Prechemotherapy laparoscopic nephrectomy for Wilms’ tumor. J Pediatr Urol 2009, 5:416–419.

    Article  CAS  PubMed  Google Scholar 

  44. Green DM, Breslow NE, Beckwith JB, et al.: Treatment with nephrectomy only for small, stage I/favorable histology Wilms’ tumor: a report from the National Wilms’ Tumor Study Group. J Clin Oncol 2001, 19:3719–3724.

    CAS  PubMed  Google Scholar 

  45. Malogolowkin M, Cotton CA, Green DM, et al.: Treatment of Wilms tumor relapsing after initial treatment with vincristine, actinomycin D, and doxorubicin. A report from the National Wilms Tumor Study Group. Pediatr Blood Cancer 2008, 50:236–241.

    Article  PubMed  Google Scholar 

  46. Kalapurakal JA, Nan B, Norkool P, et al.: Treatment outcomes in adults with favorable histologic type Wilms tumor: an update from the National Wilms Tumor Study Group. Int J Radiat Oncol Biol Phys 2004, 60:1379–1384.

    Article  PubMed  Google Scholar 

  47. de Kraker J, Graf N, van Tinteren H, et al.: Reduction of postoperative chemotherapy in children with stage I intermediate-risk and anaplastic Wilms’ tumour (SIOP 93-01 trial): a randomised controlled trial. Lancet 2004, 364:1229–1235.

    Article  PubMed  Google Scholar 

  48. Pritchard-Jones K, Kelsey A, Vujanic G, et al.: Older age is an adverse prognostic factor in stage I, favorable histology Wilms’ tumor treated with vincristine monochemotherapy: a study by the United Kingdom Children’s Cancer Study Group, Wilms’ Tumor Working Group. J Clin Oncol 2003, 21:3269–3275.

    Article  CAS  PubMed  Google Scholar 

  49. Mitchell C, Jones PM, Kelsey A, et al.: The treatment of Wilms’ tumour: results of the United Kingdom Children’s cancer study group (UKCCSG) second Wilms’ tumour study. Br J Cancer 2000, 83:602–608.

    Article  CAS  PubMed  Google Scholar 

  50. Mitchell C, Pritchard-Jones K, Shannon R, et al.: Immediate nephrectomy versus preoperative chemotherapy in the management of non-metastatic Wilms’ tumour: results of a randomised trial (UKW3) by the UK Children’s Cancer Study Group. Eur J Cancer 2006, 42:2554–2562.

    Article  CAS  PubMed  Google Scholar 

  51. • Breslow NE, Collins AJ, Ritchey ML, et al.: End stage renal disease in patients with Wilms tumor: results from the National Wilms Tumor Study Group and the United States Renal Data System. J Urol 2005, 174:1972–1975. This report from the NWTSG discusses the risks of renal failure following the treatment of WT. It provides the best estimates of renal failure risk in this population and identifies groups of patients at significant risk.

    Article  PubMed  Google Scholar 

  52. Hamilton TE, Ritchey ML, Argani P, et al.: Synchronous bilateral Wilm’s tumor with complete radiographic response managed without surgical resection: a report from the National Wilm’s Tumor Study 4. J Pediatr Surg 2008, 43:1982–1984.

    Article  PubMed  Google Scholar 

  53. Shamberger RC, Haase GM, Argani P, et al.: Bilateral Wilms’ tumors with progressive or nonresponsive disease. J Pediatr Surg 2006, 41:652–657.

    Article  PubMed  Google Scholar 

  54. • Davidoff AM, Giel DW, Jones DP, et al.: The feasibility and outcome of nephron-sparing surgery for children with bilateral Wilms tumor. The St Jude Children’s Research Hospital experience: 1999–2006. Cancer 2008, 112: 2060–2070. This article should be read by all surgeons treating patients with bilateral WT or considering renal-sparing surgery in children with renal tumor. It shows that preservation of the kidneys is possible even with apparent significant tumor burden on imaging studies.

    Article  PubMed  Google Scholar 

  55. Haecker FM, von Schweinitz D, Harms D, et al.: Partial nephrectomy for unilateral Wilms tumor: results of study SIOP 93-01/GPOH. J Urol 2003, 170:939–942.

    Article  PubMed  Google Scholar 

  56. Cozzi F, Schiavetti A, Morini F, et al.: Renal function adaptation in children with unilateral renal tumors treated with nephron sparing surgery or nephrectomy. J Urol 2005, 174:1404–1408.

    Article  PubMed  Google Scholar 

  57. Wright KD, Green DM, Daw NC: Late effects of treatment for Wilms tumor. Pediatr Hematol Oncol 2009, 26:407–413.

    PubMed  Google Scholar 

  58. Cotton CA, Peterson S, Norkool PA, et al.: Early and late mortality after diagnosis of Wilms tumor. J Clin Oncol 2009, 27:1304–1309.

    Article  PubMed  Google Scholar 

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  1. Department of Urology, Mayo Clinic Arizona, 5777 E Mayo Blvd, Phoenix, AZ, 85054, USA

    Leah Nakamura & Michael Ritchey

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  1. Leah Nakamura
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  2. Michael Ritchey
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Correspondence to Michael Ritchey.

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Nakamura, L., Ritchey, M. Current Management of Wilms’ Tumor. Curr Urol Rep 11, 58–65 (2010). https://doi.org/10.1007/s11934-009-0082-z

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