Wilms’ Tumor

  • Carlos H. Martínez
  • Sumit Dave
  • Jonathan Izawa
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 685)


Wilms’ tumor or nephroblastoma is the most frequent renal tumor in children and is associated with different congenital anomalies and syndromes. A significant number of studies in genetics and molecular biology have improved our understanding of this malignancy discovering as well how different genes play a critical role in the organogenesis process. Derived from these studies promising markers have emerged relating translational medicine with clinical prognosis, which has been traditionally defined by the histopathological changes and the tumoral stage.

During the last 40 years the therapeutic outcomes have improved after multi-centric and multidisciplinary efforts represented mainly by The National Wilms’ Tumor Study Group (currently the Renal Tumor Committee of the Children’s Oncology Group) from North America and the International Society of Paediatric Oncology from Europe and this has served as a role model for establishing similar trials for other pediatric tumors.

Our aim with this chapter of Wilms’ tumor is to present the state of knowledge in translational and clinical areas in a balanced persp


Preoperative Chemotherapy Renal Tumor Neural Cell Adhesion Molecule Tumor Rupture United States Renal Data System 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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  1. 1.
    Wilms M. Die Mischgeschwulste. I. Die Mischgeschwulste der Niere, A. Georgi, Leipzig. 1899.Google Scholar
  2. 2.
    Willetts IE. Jessop and the Wilms’ tumor. J Pediatr Surg 2003; 38:1496–1498.PubMedCrossRefGoogle Scholar
  3. 3.
    Knudson AG Jr, Strong LC. Mutation and cancer: a model for Wilms’ tumor of the kidney. J Natl Cancer Inst 1972; 48:313–324.PubMedGoogle Scholar
  4. 4.
    The Canadian Cancer Society/National Cancer Institute of Canada. Canadian Cancer Statistics 2008. Chidhood cancer in ages 0 to 14 2008; 2009.Google Scholar
  5. 5.
    National Cancer Institute. SEER Cancer Statistics Review 1975-2005. ICCC. 04/09/08; 2009.Google Scholar
  6. 6.
    Ton CC, Hirvonen H, Miwa H et al. Positional cloning and characterization of a paired box-and homeobox-containing gene from the aniridia region. Cell 1991; 67:1059–1074.PubMedCrossRefGoogle Scholar
  7. 7.
    Call KM, Glaser T, Ito CY et al. Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms’ tumor locus. Cell 1990; 60:509–520.PubMedCrossRefGoogle Scholar
  8. 8.
    Hohenstein P, Hastie ND. The many facets of the Wilms’ tumour gene, WT1. Hum Mol Genet 2006; 15 Spec No 2:R196–201.PubMedCrossRefGoogle Scholar
  9. 9.
    Dutton JR, Lahiri D, Ward A. Different isoforms of the Wilms’ tumour protein WT1 have distinct patterns of distribution and trafficking within the nucleus. Cell Prolif 2006; 39:519–535.PubMedCrossRefGoogle Scholar
  10. 10.
    Natoli TA, McDonald A, Alberta JA et al. A mammal-specific exon of WT1 is not required for development or fertility. Mol Cell Biol 2002; 22:4433–4438.PubMedCrossRefGoogle Scholar
  11. 11.
    Scharnhorst V, van der Eb AJ, Jochemsen AG. WT1 proteins: functions in growth and differentiation. Gene 2001; 273:141–161.PubMedCrossRefGoogle Scholar
  12. 12.
    Wagner KJ, Roberts SG. Transcriptional regulation by the Wilms’ tumour suppressor protein WT1. Biochem Soc Trans 2004; 32:932–935.PubMedCrossRefGoogle Scholar
  13. 13.
    Rivera MN, Haber DA. Wilms’ tumour: connecting tumorigenesis and organ development in the kidney. Nat Rev Cancer 2005; 5:699–712.PubMedCrossRefGoogle Scholar
  14. 14.
    Kreidberg JA, Sariola H, Loring JM et al. WT-1 is required for early kidney development. Cell 1993; 74:679–691.PubMedCrossRefGoogle Scholar
  15. 15.
    Kreidberg JA, Hartwig S. Wilms’ tumor-1: a riddle wrapped in a mystery, inside a kidney. Kidney Int 2008; 74:411–412.PubMedCrossRefGoogle Scholar
  16. 16.
    Morrison AA, Viney RL, Saleem MA et al. New insights into the function of the Wilms tumor suppressor gene WT1 in podocytes. Am J Physiol Renal Physiol 2008; 295:F12–7.PubMedCrossRefGoogle Scholar
  17. 17.
    Steege A, Fahling M, Paliege A et al. Wilms’ tumor protein (-KTS) modulates renin gene transcription. Kidney Int 2008; 74:458–466.PubMedCrossRefGoogle Scholar
  18. 18.
    Ehrlich PF. Wilms tumor: progress and considerations for the surgeon. Surg Oncol 2007; 16:157–171.PubMedCrossRefGoogle Scholar
  19. 19.
    Karnik P, Chen P, Paris M et al. Loss of heterozygosity at chromosome 11p15 in Wilms tumors: identification of two independent regions. Oncogene 1998; 17:237–240.PubMedCrossRefGoogle Scholar
  20. 20.
    Sparago A, Russo S, Cerrato F et al. Mechanisms causing imprinting defects in familial Beckwith-Wiedemann syndrome with Wilms’ tumour. Hum Mol Genet 2007; 16:254–264.PubMedCrossRefGoogle Scholar
  21. 21.
    Scott RH, Douglas J, Baskcomb L et al. Constitutional 11p15 abnormalities, including heritable imprinting center mutations, cause nonsyndromic Wilms tumor. Nat Genet 2008; 40:1329–1334.PubMedCrossRefGoogle Scholar
  22. 22.
    Safford SD, Goyeau D, Freemerman AJ et al. Fine mapping of Wilms’ tumors with 16q loss of heterozygosity localizes the putative tumor suppressor gene to a region of 6.7 megabases. Ann Surg Oncol 2003; 10:136–143.PubMedCrossRefGoogle Scholar
  23. 23.
    Guertl B, Ratschek M, Harms D et al. Clonality and loss of heterozygosity of WT genes are early events in the pathogenesis of nephroblastomas. Hum Pathol 2003; 34:278–281.PubMedCrossRefGoogle Scholar
  24. 24.
    Rivera MN, Kim WJ, Wells J et al. An X chromosome gene, WTX, is commonly inactivated in Wilms tumor. Science 2007; 315:642–645.PubMedCrossRefGoogle Scholar
  25. 25.
    Waber PG, Chen J, Nisen PD. Infrequency of ras, p53, WT1, or RB gene alterations in Wilms tumors. Cancer 1993; 72:3732–3738.PubMedCrossRefGoogle Scholar
  26. 26.
    Bardeesy N, Falkoff D, Petruzzi MJ et al. Anaplastic Wilms’ tumour, a subtype displaying poor prognosis, harbours p53 gene mutations. Nat Genet 1994; 7:91–97.PubMedCrossRefGoogle Scholar
  27. 27.
    Ghanem MA, Van der Kwast TH, Den Hollander JC et al. The prognostic significance of apoptosis-associated proteins BCL-2, BAX and BCL-X in clinical nephroblastoma. Br J Cancer 2001; 85:1557–1563.PubMedCrossRefGoogle Scholar
  28. 28.
    Re GG, Hazen-Martin DJ, El Bahtimi R et al. Prognostic significance of Bcl-2 in Wilms’ tumor and oncogenic potential of Bcl-X(L) in rare tumor cases. Int J Cancer 1999; 84:192–200.PubMedCrossRefGoogle Scholar
  29. 29.
    Su MC, Huang WC, Lien HC. Beta-catenin expression and mutation in adult and pediatric Wilms’ tumors. APMIS 2008; 116:771–778.PubMedCrossRefGoogle Scholar
  30. 30.
    Maiti S, Alam R, Amos CI et al. Frequent association of beta-catenin and WT1 mutations in Wilms tumors. Cancer Res 2000; 60:6288–6292.PubMedGoogle Scholar
  31. 31.
    Koesters R, Ridder R, Kopp-Schneider A et al. Mutational activation of the beta-catenin proto-oncogene is a common event in the development of Wilms’ tumors. Cancer Res 1999; 59:3880–3882.PubMedGoogle Scholar
  32. 32.
    Ghanem MA, van Steenbrugge GJ, Nijman RJ et al. Prognostic markers in nephroblastoma (Wilms’ tumor). Urology 2005; 65:1047–1054.PubMedCrossRefGoogle Scholar
  33. 33.
    Nowicki M, Ostalska-Nowicka D, Kaczmarek M et al. The significance of VEGF-C/VEGFR-2 interaction in the neovascularization and prognosis of nephroblastoma (Wilms’ tumour). Histopathology 2007; 50:358–364.PubMedCrossRefGoogle Scholar
  34. 34.
    Pode-Shakked N, Metsuyanim S, Rom-Gross E et al. Developmental Tumorigenesis: NCAM as a putative marker for the malignant renal stem/progenitor cell population. J Cell Mol Med 2008;.Google Scholar
  35. 35.
    Ghanem MA, Van Steenbrugge GJ, Van Der Kwast TH et al. Expression and prognostic value of CD44 isoforms in nephroblastoma (Wilms tumor). J Urol 2002; 168:681–686.PubMedCrossRefGoogle Scholar
  36. 36.
    Lindop GB, Duncan K, Millan DW et al. Renin gene expression in nephroblastoma. J Pathol 1990; 161:93–97.PubMedCrossRefGoogle Scholar
  37. 37.
    McKenzie KJ, Ferrier RK, Howatson AG et al. Demonstration of renin gene expression in nephroblastoma by in situ hybridization. J Pathol 1996; 180:71–73.PubMedCrossRefGoogle Scholar
  38. 38.
    Mason JE, Goodfellow PJ, Grundy PE et al 16q loss of heterozygosity and microsatellite instability in Wilms’ tumor. J Pediatr Surg 2000; 35:891–6; discussion 896-7.PubMedCrossRefGoogle Scholar
  39. 39.
    Idikio HA. Expression of DNA mismatch repair proteins hMSH2 and hMLH1 and the cyclin G1 inhibitor, p21(waf1/cip1) in pediatric tumors: correlation with response to therapy. Oncol Rep 2001; 8:965–971.PubMedGoogle Scholar
  40. 40.
    Breslow NE, Olson J, Moksness J et al. Familial Wilms’ tumor: a descriptive study. Med Pediatr Oncol 1996; 27:398–403.PubMedCrossRefGoogle Scholar
  41. 41.
    Rapley EA, Barfoot R, Bonaiti-Pellie C et al. Evidence for susceptibility genes to familial Wilms tumour in addition to WT1, FWT1 and FWT2. Br J Cancer 2000; 83:177–183.PubMedCrossRefGoogle Scholar
  42. 42.
    Weksberg R, Shuman C, Smith AC. Beckwith-Wiedemann syndrome. Am J Med Genet C Semin Med Genet 2005; 137C:12–23.PubMedCrossRefGoogle Scholar
  43. 43.
    Rump P, Zeegers MP, van Essen AJ. Tumor risk in Beckwith-Wiedemann syndrome: A review and meta-analysis. Am J Med Genet A 2005; 136:95–104.PubMedGoogle Scholar
  44. 44.
    Bliek J, Gicquel C, Maas S et al. Epigenotyping as a tool for the prediction of tumor risk and tumor type in patients with Beckwith-Wiedemann syndrome (BWS). J Pediatr 2004; 145:796–799.PubMedCrossRefGoogle Scholar
  45. 45.
    Miller RW, Fraumeni JF, Jr, Manning MD. Association of Wilms’s Tumor with Aniridia, Hemihypertrophy and Other Congenital Malformations. N Engl J Med 1964; 270:922–927.PubMedCrossRefGoogle Scholar
  46. 46.
    Muto R, Yamamori S, Ohashi H et al. Prediction by FISH analysis of the occurrence of Wilms tumor in aniridia patients. Am J Med Genet 2002; 108:285–289.PubMedCrossRefGoogle Scholar
  47. 47.
    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.PubMedCrossRefGoogle Scholar
  48. 48.
    Denys P, Malvaux P, Van Den Berghe H et al. Association of an anatomo-pathological syndrome of male pseudohermaphroditism, Wilms’ tumor, parenchymatous nephropathy and XX/XY mosaicism. Arch Fr Pediatr 1967; 24:729–739.PubMedGoogle Scholar
  49. 49.
    Drash A, Sherman F, Hartmann WH et al. A syndrome of pseudohermaphroditism, Wilms’ tumor, hypertension and degenerative renal disease. J Pediatr 1970; 76:585–593.PubMedCrossRefGoogle Scholar
  50. 50.
    Pelletier J, Bruening W, Kashtan CE et al. Germline mutations in the Wilms’ tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome. Cell 1991; 67:437–447.PubMedCrossRefGoogle Scholar
  51. 51.
    Viney RL, Morrison AA, van den Heuvel LP et al. A proteomic investigation of glomerular podocytes from a Denys-Drash syndrome patient with a mutation in the Wilms tumour suppressor gene WT1. Proteomics 2007; 7:804–815.PubMedCrossRefGoogle Scholar
  52. 52.
    Habib R, Loirat C, Gubler MC et al. The nephropathy associated with male pseudohermaphroditism and Wilms’ tumor (Drash syndrome): a distinctive glomerular lesion—report of 10 cases. Clin Nephrol 1985; 24:269–278.PubMedGoogle Scholar
  53. 53.
    Perlman M, Goldberg GM, Bar-Ziv J et al. Renal hamartomas and nephroblastomatosis with fetal gigantism: a familial syndrome. J Pediatr 1973; 83:414–418.PubMedCrossRefGoogle Scholar
  54. 54.
    Perlman M, Levin M, Wittels B. Syndrome of fetal gigantism, renal hamartomas and nephroblastomatosis with Wilms’ tumor. Cancer 1975; 35:1212–1217.PubMedCrossRefGoogle Scholar
  55. 55.
    Piccione M, Cecconi M, Giuffre M et al. Perlman syndrome: clinical report and nine-year follow-up. Am J Med Genet A 2005; 139A:131–135.PubMedCrossRefGoogle Scholar
  56. 56.
    Alessandri JL, Cuillier F, Ramful D et al. Perlman syndrome: report, prenatal findings and review. Am J Med Genet A 2008; 146A:2532–2537.PubMedCrossRefGoogle Scholar
  57. 57.
    Govender D. The Pathology of Nephroblastoma. Current Diagnostic Pathology. 2000; 6:45–54.CrossRefGoogle Scholar
  58. 58.
    Perlman EJ. Pediatric renal tumors: practical updates for the pathologist. Pediatr Dev Pathol 2005; 8:320–338.PubMedCrossRefGoogle Scholar
  59. 59.
    Sebire NJ, Vujanic GM. Paediatric renal tumours: recent developments, new entities and pathological features. Histopathology 2009; 54:516–528.PubMedCrossRefGoogle Scholar
  60. 60.
    Faria P, Beckwith JB, Mishra K et al. Focal versus diffuse anaplasia in Wilms tumor—new definitions with prognostic significance: a report from the National Wilms Tumor Study Group. Am J Surg Pathol 1996; 20:909–920.PubMedCrossRefGoogle Scholar
  61. 61.
    Vujanic GM, Harms D, Sandstedt B et al. New definitions of focal and diffuse anaplasia in Wilms tumor: the International Society of Paediatric Oncology (SIOP) experience. Med Pediatr Oncol 1999; 32:317–323.PubMedCrossRefGoogle Scholar
  62. 62.
    Vujanic GM, Sandstedt B, Harms D et al. Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol 2002; 38:79–82.PubMedCrossRefGoogle Scholar
  63. 63.
    Beckwith JB, Kiviat NB, Bonadio JF. Nephrogenic rests, nephroblastomatosis and the pathogenesis of Wilms’ tumor. Pediatr Pathol 1990; 10:1–36.PubMedCrossRefGoogle Scholar
  64. 64.
    Beckwith JB. Nephrogenic rests and the pathogenesis of Wilms tumor: developmental and clinical considerations. Am J Med Genet 1998; 79:268–273.PubMedCrossRefGoogle Scholar
  65. 65.
    Hennigar RA, O’Shea PA, Grattan-Smith JD. Clinicopathologic features of nephrogenic rests and nephroblastomatosis. Adv Anat Pathol 2001; 8:276–289.PubMedCrossRefGoogle Scholar
  66. 66.
    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.PubMedCrossRefGoogle Scholar
  67. 67.
    Perlman EJ, Faria P, Soares A et al. Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients. Pediatr Blood Cancer 2006; 46:203–221.PubMedCrossRefGoogle Scholar
  68. 68.
    Varan A. Wilms’ tumor in children: an overview. Nephron Clin Pract 2008; 108:c83–90.PubMedCrossRefGoogle Scholar
  69. 69.
    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.PubMedCrossRefGoogle Scholar
  70. 70.
    Ritchey ML, Kelalis PP, Breslow N et al. Intracaval and atrial involvement with nephroblastoma: review of National Wilms Tumor Study-3. J Urol 1988; 140:1113–1118.PubMedGoogle Scholar
  71. 71.
    Baxter PA, Nuchtern JG, Guillerman RP et al. Acquired von Willebrand syndrome and Wilms tumor: not always benign. Pediatr Blood Cancer 2009; 52:392–394.PubMedCrossRefGoogle Scholar
  72. 72.
    Granger J, Gidvani VK. Acquired factor VII deficiency associated with Wilms tumor. Pediatr Blood Cancer 2009; 52:394–395.PubMedCrossRefGoogle Scholar
  73. 73.
    Wang J, Zhang G. Paraneoplastic Cushing syndrome because of corticotrophin-releasing hormone-secreting Wilms’ tumor. J Pediatr Surg 2008; 43:2099–2101.PubMedCrossRefGoogle Scholar
  74. 74.
    Riccabona M. Imaging of renal tumours in infancy and childhood. Eur Radiol 2003; 13(Suppl 4):L116–29.PubMedCrossRefGoogle Scholar
  75. 75.
    McHugh K. Renal and adrenal tumours in children. Cancer Imaging 2007; 7:41–51.PubMedCrossRefGoogle Scholar
  76. 76.
    Tan TY, Amor DJ. Tumour surveillance in Beckwith-Wiedemann syndrome and hemihyperplasia: a critical review of the evidence and suggested guidelines for local practice. J Paediatr Child Health 2006; 42:486–490.PubMedCrossRefGoogle Scholar
  77. 77.
    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.PubMedCrossRefGoogle Scholar
  78. 78.
    Meisel JA, Guthrie KA, Breslow NE et al. Significance and management of computed tomography detected pulmonary nodules: a report from the National Wilms Tumor Study Group. Int J Radiat Oncol Biol Phys 1999; 44:579–585.PubMedCrossRefGoogle Scholar
  79. 79.
    Owens CM, Veys PA, Pritchard J et al. Role of chest computed tomography at diagnosis in the management of Wilms’ tumor: a study by the United Kingdom Children’s Cancer Study Group. J Clin Oncol 2002; 20:2768–2773.PubMedCrossRefGoogle Scholar
  80. 80.
    Martinez CH, Chalasani V, Knudsen B et al. Virtual Reality May Improve Training of Renal Surgeons. SPIE Newsroom 2009.Google Scholar
  81. 81.
    Gylys-Morin V, Hoffer FA, Kozakewich H et al. Wilms tumor and nephroblastomatosis: imaging characteristics at gadolinium-enhanced MR imaging. Radiology 1993; 188:517–521.PubMedGoogle Scholar
  82. 82.
    Shah NB, Platt SL. ALARA: is there a cause for alarm? Reducing radiation risks from computed tomography scanning in children. Curr Opin Pediatr 2008; 20:243–247.PubMedCrossRefGoogle Scholar
  83. 83.
    Kalapurakal JA, Dome JS, Perlman EJ et al. Management of Wilms’ tumour: current practice and future goals. Lancet Oncol 2004; 5:37–46.PubMedCrossRefGoogle Scholar
  84. 84.
    D’Angio GJ. The National Wilms Tumor Study: a 40 year perspective. Lifetime Data Anal 2007; 13:463–470.PubMedCrossRefGoogle Scholar
  85. 85.
    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.PubMedGoogle Scholar
  86. 86.
    Burger D, Moorman-Voestermans CG, Mildenberger H et al. The advantages of preoperative therapy in Wilms’ tumour. A summarised report on clinical trials conducted by the International Society of Paediatric Oncology (SIOP). Z Kinderchir 1985; 40:170–175.PubMedGoogle Scholar
  87. 87.
    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.PubMedCrossRefGoogle Scholar
  88. 88.
    Moorman-Voestermans CG, Aronson DC, Staalman CR et al. Is partial nephrectomy appropriate treatment for unilateral Wilms’ tumor? J Pediatr Surg 1998; 33:165–170.PubMedCrossRefGoogle Scholar
  89. 89.
    Zani A, Schiavetti A, Gambino M et al. Long-term outcome of nephron sparing surgery and simple nephrectomy for unilateral localized Wilms tumor. J Urol 2005; 173:946–8; discussion 948.PubMedCrossRefGoogle Scholar
  90. 90.
    D’Angio GJ, Evans AE, Breslow N et al. The treatment of Wilms’ tumor: Results of the national Wilms’ tumor study. Cancer 1976; 38:633–646.PubMedCrossRefGoogle Scholar
  91. 91.
    D’Angio GJ, Evans A, Breslow N et al. The treatment of Wilms’ tumor: results of the Second National Wilms’ Tumor Study. Cancer 1981; 47:2302–2311.PubMedCrossRefGoogle Scholar
  92. 92.
    D’Angio GJ, Breslow N, Beckwith JB et al. Treatment of Wilms’ tumor. Results of the Third National Wilms’ Tumor Study. Cancer 1989; 64:349–360.PubMedCrossRefGoogle Scholar
  93. 93.
    Green DM, Breslow NE, Beckwith JB et al. Comparison between single-dose and divided-dose administration of dactinomycin and doxorubicin for patients with Wilms’ tumor: a report from the National Wilms’ Tumor Study Group. J Clin Oncol 1998; 16:237–245.PubMedGoogle Scholar
  94. 94.
    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.PubMedCrossRefGoogle Scholar
  95. 95.
    Tournade MF, Com-Nougue C, Voute PA et al. Results of the Sixth International Society of Pediatric Oncology Wilms’ Tumor Trial and Study: a risk-adapted therapeutic approach in Wilms’ tumor. J Clin Oncol 1993; 11:1014–1023.PubMedGoogle Scholar
  96. 96.
    Lemerle J, Voute PA, Tournade MF et al. Preoperative versus postoperative radiotherapy, single versus multiple courses of actinomycin D, in the treatment of Wilms’ tumor. Preliminary results of a controlled clinical trial conducted by the International Society of Paediatric Oncology (S.I.O.P.). Cancer 1976; 38:647–654.PubMedCrossRefGoogle Scholar
  97. 97.
    Graf N, Tournade MF, de Kraker J. The role of preoperative chemotherapy in the management of Wilms’ tumor. The SIOP studies. International Society of Pediatric Oncology. Urol Clin North Am 2000; 27:443–454.PubMedCrossRefGoogle Scholar
  98. 98.
    Lemerle J, Voute PA, Tournade MF et al. Effectiveness of preoperative chemotherapy in Wilms’ tumor: results of an International Society of Paediatric Oncology (SIOP) clinical trial. J Clin Oncol 1983; 1:604–609.PubMedGoogle Scholar
  99. 99.
    Tournade MF, Com-Nougue C, de Kraker J et al. ai]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.PubMedGoogle Scholar
  100. 100.
    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.PubMedCrossRefGoogle Scholar
  101. 101.
    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.PubMedCrossRefGoogle Scholar
  102. 102.
    Godzinski J, Tournade MF, deKraker 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.PubMedCrossRefGoogle Scholar
  103. 103.
    de Kraker J, Delemarre JF, Lilien MR et al. Misstaging in nephroblastoma. Causes and consequences. A report of the Sixth Nephroblastoma Trial and Study of the International Society of Paediatric Oncology. Eur J Pediatr Surg 1999; 9:153–157.PubMedCrossRefGoogle Scholar
  104. 104.
    Leape LL, Breslow NE, Bishop HC. The surgical treatment of Wilms’ tumor: results of the National Wilms’ Tumor Study. Ann Surg 1978; 187:351–356.PubMedCrossRefGoogle Scholar
  105. 105.
    Mitchell C, Pritchard-Jones K, Shannon R et al. Immediate nephrectomy versus preoperative chemotherapy in the management of nonmetastatic Wilms’ tumour: results of a randomised trial (UKW3) by the UK Children’s Cancer Study Group. Eur J Cancer 2006; 42:2554–2562.PubMedCrossRefGoogle Scholar
  106. 106.
    Fuchs J, Kienecker K, Furtwangler R et al. Surgical aspects in the treatment of patients with unilateral wilms tumor: a report from the SIOP 93-01/German Society of Pediatric Oncology and Hematology. Ann Surg 2009; 249:666–671.PubMedCrossRefGoogle Scholar
  107. 107.
    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.PubMedCrossRefGoogle Scholar
  108. 108.
    Horwitz JR, Ritchey ML, Moksness J et al. Renal salvage procedures in patients with synchronous bilateral Wilms’ tumors: a report from the National Wilms’ Tumor Study Group. J Pediatr Surg 1996; 31:1020–1025.PubMedCrossRefGoogle Scholar
  109. 109.
    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.PubMedCrossRefGoogle Scholar
  110. 110.
    Izawa JI, Al-Omar M, Winquist E et al. Prognostic variables in adult Wilms tumour. Can J Surg 2008; 51:252–256.PubMedGoogle Scholar

Copyright information

© Landes Bioscience and Springer Science+Business Media 2010

Authors and Affiliations

  • Carlos H. Martínez
    • 1
  • Sumit Dave
    • 2
  • Jonathan Izawa
    • 3
  1. 1.Department of Surgery Division of Urology Schulich School of Medicine & DentistryThe University of Western Ontario London Health Sciences Centre-Victoria HospitalLondonCanada
  2. 2.Departments of Surgery & Pediatrics Division of Urology Schulich School of Medicine & DentistryThe University of Western Ontario London Health Sciences Centre-Victoria HospitalLondonCanada
  3. 3.Departments of Surgery & Oncology Divisions of Surgical Oncology & Urology Schulich School of Medicine & DentistryThe University of Western Ontario London Health Sciences Centre-Victoria HospitalLondonCanada

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