Recent advances and controversies in the management of Wilms’ tumor

  • Ellen Shapiro
Part of the Cancer Treatment and Research book series (CTAR, volume 46)


Over the past 30 years, there has been significant improvement in the survival of patients with Wilms’ tumor. Cure rates have improved due to the multitherapeutic approach to the management of the tumors. Combined surgical extirpation and chemotherapy now allow for greater than 90% survival in patients with stage I disease [1]. Chemotherapy, radiation, and second-look surgical procedures can be successfully used in patients presenting with metastatic disease or extensive local disease involving adjacent organs or bilateral renal involvement.


Preoperative Chemotherapy Tumor Study Contralateral Kidney Clear Cell Sarcoma Malignant Rhabdoid Tumor 
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  1. 1.
    D’Angio GJ, Breslow N, Beckwith B, Evans A, Baum E, deLorimier A, Fernbach D, Hrabovsky E, Jones B, Kelalis P, Otherson HB, Tefft M, Thomas PRM: The treatment of Wilms’ tumor: Results of the Third National Wilms’ Tumor Study. Cancer, in press.Google Scholar
  2. 2.
    Breslow NE, Palmer NF, Hill LR, Buring J, D’Angio GJ Wilms’ tumor: Prognostic factors for patients without metastases at diagnosis; Results of the National Wilms’ Tumor Study. Cancer 41: 1577, 1978.PubMedCrossRefGoogle Scholar
  3. 3.
    D’Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Feigl P, Goodwin W Leape LL, Sinks LF, Sutow W, Tefft M, Wolff J: The treatment of Wilms’ tumor: Results of the National Wilms’ Tumor Study. Cancer 38: 633, 1976.PubMedCrossRefGoogle Scholar
  4. 4.
    D’Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Farewell V, Goodwin W, Leape L, Palmer N, Sinks L, Sutow W, Tefft M, Wolff J: The treatment of Wilms’ tumor: Results of the Second National Wilms’ Tumor Study. Cancer 47: 2302, 1981.PubMedCrossRefGoogle Scholar
  5. 5.
    Breslow N, Chruchill G, Beckwith JB, Fernbach DJ, Otherson HB, Tefft M, D’Angio GJ: Prognosis for Wilms’ tumor patients with nonmetastatic disease at diagnosis: Results of the Second National Wilms’ Tumor Study. J Clin Oncol 3: 521, 1985.PubMedGoogle Scholar
  6. 6.
    National Wilms’ Tumor Study-4 Therapeutic Trial protocol. November, 1987.Google Scholar
  7. 7.
    Beckwith JB: Wilms’ tumor and other renal tumors of childhood. Hum Pathol 14: 481, 1983.PubMedCrossRefGoogle Scholar
  8. 8.
    Beckwith JB, Palmer NF: Histopathology and prognosis of Wilms’ tumor. Cancer 41: 1937, 1978.PubMedCrossRefGoogle Scholar
  9. 9.
    Weeks DA, Beckwith JB, Mierau GA: Rhabdoid tumor of kidney: The National Wilms’ Tumor Study Experience. Lab Invest 58: 101A, 1988.Google Scholar
  10. 10.
    Tsuneyoshi M, Daimaru Y, Hashimoto H, Enjoji M: Malignant soft tissue neoplasms with the histologic features of renal rhabdoid tumours: An ultrastructural and immunohistoche-mical study. Hum Path 16: 1235, 1985.PubMedCrossRefGoogle Scholar
  11. 11.
    Rousseau-Merck MF, Nogues C, Nezelof C, Marin-Cudraz B, Paulin D: Infantile renal tumors associated with hypercalcemia. Characterization of intermediate-filament clusters. Arch Patho Lab Med 107: 311, 1983.Google Scholar
  12. 12.
    Mayes LC, Kasselberg AG Roloff JS, Lukens JN: Hypercalcemia associated with immu-noreactive parathyroid hormone in a malignant rhabdoid tumor of the kidney (rhabdoid Wilms’ tumor). Cancer 54: 882, 1984.PubMedCrossRefGoogle Scholar
  13. 13.
    Bonnin JM, Rubinstein LJ, Palmer NF Beckwith JB: The association of embryonal tumors originating in the kidney and in the brain: A report of seven cases. Cancer 54: 2137, 1984.PubMedCrossRefGoogle Scholar
  14. 14.
    Beckwith JB: Wilms’ tumor and other renal tumors of childhood: An update. J Urol 136: 320, 1986.PubMedGoogle Scholar
  15. 15.
    Pysher TJ, Beckwith JB: Clear cell sarcoma of the kidney (CCSK): Analysis of 82 cases from the Second and Third National Wilms’ Tumor Studies. Lab Invest 58: 73A, 1988.Google Scholar
  16. 16.
    Farewell VT, D’Angio GJ, Breslow N, Norkook P: Retrospective validation of a new staging system for Wilms’ tumor. Cancer Clin Trials 4: 167, 1981.PubMedGoogle Scholar
  17. 17.
    Carli M, Pastore G, Paolucci G, et al.: High single doses versus 5 day divided doses of dactinomycin (AMD) in childhood rhabdomyosarcoma. Preliminary results. [Abstract] Proc ASCO 2: 76, 1983.Google Scholar
  18. 18.
    Blatt J, Trigg ME, Pizzo PA, et al.: Tolerance to single-dose dactinomycin in combination chemotherapy for solid tumors. Cancer Treat Rep 65: 145, 1981.PubMedGoogle Scholar
  19. 19.
    Green DM, Finklestein JZ, Norkook P, D’Angio GJ: Severe hepatic toxicity after treatment with single-dose dactinomycin and vincristine: A report of the National Wilms’ Tumor Study. Cancer 62: 270, 1988.PubMedCrossRefGoogle Scholar
  20. 20.
    Snyder HM, D’Angio GJ, Evans AE, Raney RB: Pediatric oncology. In: Campbell’s Urology, 5th ed., PC Walsh, RF Gittes, AD Perlmutter, TA Stamey (eds). WB Saunders, Philadelphia, 1986.Google Scholar
  21. 21.
    Reiman T, Siegel MJ, Shackelford GD: Wilms’ tumor in children: Abdominal CT and US evaluation. Radiology 160: 501, 1986.PubMedGoogle Scholar
  22. 22.
    Clayman RV, Sheldon CA, Gonzalez R: Wilms’ tumor: An approach to vena cavai intrusion. Prog Ped Surg 15: 285, 1982.Google Scholar
  23. 23.
    Nakayama DK, deLorimier AA, O’Neil JA, Norkool P, D’Angio GJ: Intracardiac extension of Wilms’ tumor: A report of the National Wilms’ Tumor Study. Ann Surg 204: 693, 1986.PubMedCrossRefGoogle Scholar
  24. 24.
    Wilimas JA, Douglass EC, Magel HL, Fitch S, Hustu HO: Significance of pulmonary computed tomography at diagnosis in Wilms’ tumor. J Clin Oncol 6: 1144, 1988.PubMedGoogle Scholar
  25. 25.
    Pagano F, Pennelli N: Ureteral and vesical metastases in nephroblastoma. Br J Urol 46: 409, 1974.PubMedCrossRefGoogle Scholar
  26. 26.
    Taykurt A: Wilms’ tumor at lower end of the ureter extending to the bladder: Case report. J Urol 107: 142, 1972.PubMedGoogle Scholar
  27. 27.
    Cremin BJ: Non-function in nephroblastoma (Wilms’ tumor): A report on the excretory urography of nine cases. Clin Rad 30: 197, 1979.CrossRefGoogle Scholar
  28. 28.
    Canty TG, Nagaraj HS, Shearer LS: Nonvisualization of the intravenous pyelogram — a poor prognostic sign in Wilms’ tumor? J Ped Surg 14: 825, 1979.CrossRefGoogle Scholar
  29. 29.
    Stevens PS, Eckstein HB: Ureteral metastasis from Wilms’ tumor. J Urol 115: 467, 1976.PubMedGoogle Scholar
  30. 30.
    Waggert J, Koop CE: Wilms’ tumor: Preoperative radiotherapy and chemotherapy in the management of massive tumors. Cancer 26: 338, 1970.CrossRefGoogle Scholar
  31. 31.
    Bracken RB, Sutow WW, Jaffe N, Ayala A, Guarda L: Preoperative chemotherapy for Wilms’ tumor. Urology 19: 55, 1982.PubMedCrossRefGoogle Scholar
  32. 32.
    Kogan SJ, Marons H, Santorneau M, Schneider K, Reda E, Levitt SB: Successful treatment of renal vein and vena cavai extension of nephroblastoma by preoperative chemotherapy. J Urol 136: 312, 1986.PubMedGoogle Scholar
  33. 33.
    Bray GL, Pendergrass TW, Schaller RT, Kiviat N, Beckwith JB: Preoperative chemotherapy in the treatment of Wilms’ tumor diagnosed with the aid of fine needle aspiration biopsy. Am J Pediatr Hematol Oncol 8: 75, 1986.PubMedCrossRefGoogle Scholar
  34. 34.
    Lemerle J, Voute PA, Tournade MF: Effectiveness of preoperative chemotherapy in Wilms’ tumor: Results of an International Society of Pediatric Oncology (SIOP) clinical trial. J Clin Oncol 1: 640, 1983.Google Scholar
  35. 35.
    Zucker JM, Tournade MF, Voute PA, Delemarre JFM, deKraker J, Lemerle J Perry HJM, Rey A, Ducourtieux M, Com Nougue C: Report on SIOP-6 nephroblastoma trials. Proc of SIOP 31–34, 1986.Google Scholar
  36. 36.
    SIOP Nephroblastoma Trial, No. 9.Google Scholar
  37. 37.
    Beckwitn JB, Douglass EC, Rich M, Nisen P, Voute PA, Delemarre JFM, deKraker J, Ritchey ML, Kelalis PO, Arrigo S: Wilms’ tumor update: Current issues in management. Dialogues in Pediatric Urology, November, 1988.Google Scholar
  38. 38.
    Zuppan C, Beckwith B: Pretreated anaplastic Wilms’ tumor. Lab Invest 58: 108A, 1988.Google Scholar
  39. 39.
    Blute ML, Kelalis PP, Offord KP, Breslow N, Beckwith JB, D’Angio GJ: Bilateral Wilms’ tumor. J Urol 138: 968, 1987.PubMedGoogle Scholar
  40. 40.
    Kelalis PP: Wilms’ tumor, [editorial] J Urol 135: 989, 1986.PubMedGoogle Scholar
  41. 41.
    Weeks DA, Beckwith JB, Luckey DW: Relapse-associated variables in Stage 1 favorable histology Wilms’ tumor: A report of the National Wilms’ Tumor Study. Cancer 60: 1204, 1987.PubMedCrossRefGoogle Scholar
  42. 42.
    Breslow NE, Churchill G, Nesmith B, Thomas PRM, Beckwith JB, Otherson HB, D’Angio GJ: Clinicopathologic features and prognosis for Wilms’ tumor patients with metastases at diagnosis. Cancer 58: 2501, 1986.PubMedCrossRefGoogle Scholar
  43. 43.
    Thomas PRM, Shochat SJ, Norkook P, Breslow NE, D’Angio GJ: Prognostic implications of extension, invasion, or metastasis to the liver at diagnosis of Wilms’ tumor. Proc of ASCO 7: 255, 1988.Google Scholar
  44. 44.
    Maizels M, Wacksman J, Belman AB, Colodny AH: Management of Multicystic Dysplastic Kidney. Dialogues in Pediatric Urology, June, 1987.Google Scholar
  45. 45.
    Meredith Campbell Lecture. Nephrogenic rests, nephroblastomatosis, and Wilms’ Tumor, J. Bruce Beckwith, AUA 83rd Annual Meeting, Boston, MA June 3, 1988.Google Scholar
  46. 46.
    Thomas PRM, Griffith KD, Fineberg BB, Perez CA, Land VJ: Late effects of treatment for Wilms’ tumor. J Radiation Oncol Biol Phy 9: 651, 1983.CrossRefGoogle Scholar
  47. 47.
    D’Angio GJ: The child cured of cancer: A problem for the internist. Semin Oncol 9: 143, 1982.PubMedGoogle Scholar
  48. 48.
    Jaffe N, McNeese M, Mayfield JK, Riseborough EJ: Childhood urologie cancer therapy related sequelae and their impact on management. Cancer 45: 1815, 1980.PubMedGoogle Scholar
  49. 49.
    Ganick DJ: Wilms’ tumor. Hematology/Oncology Clinics North Am 1: 695, 1987.Google Scholar
  50. 50.
    Clausen N: Late recurrence of Wilms’ tumor. Med Pediatr Oncol 10: 557, 1982.PubMedCrossRefGoogle Scholar
  51. 51.
    Johnson FL, Ballis FM: Hepatopathy following irradiation and chemotherapy for Wilms’ tumor. Am J Pediatr Hematol Oncol 4: 217, 1982.PubMedGoogle Scholar
  52. 52.
    Barnard JA, Marshall GS, Neblett WW, Gray G, Grinshan FK: Noncirrhotic portal fibrosis after Wilms’ tumor therapy. Gastroenterology 90: 1054, 1986.PubMedGoogle Scholar
  53. 53.
    Mitus A, Teftt M Fellers FX: Long-term follow-up of renal functions of 108 children who underwent nephrectomy for malignant disease. Pediatrics 44: 912, 1969.Google Scholar
  54. 54.
    O’Malley B, D’Angio GJ, Vawter GF: Effects of roentgen therapy given in infancy. Am J Roentgenol 89: 1067, 1963.Google Scholar
  55. 55.
    Wikstad I, Petterson BA, Elinder G, Sokucu S, Aperia A: A comperative study of size and function of the remnant kidney in patients nephrectomized in childhood for Wilms’ tumor and hydronephrosis. Acta Pediatr Scand 75: 408, 1986.CrossRefGoogle Scholar
  56. 56.
    Dinkel E, Britscho J, Dittrich, M, Schulte-Wissermann H, Ertel M: Renal growth in patients nephrectomized for Wilms’ tumor as compared to renal agenesis. Eur J Pediatr 147: 54, 1988.PubMedCrossRefGoogle Scholar
  57. 57.
    Breslow NE, Norkool PA, Olshan A, Evans A, D’Angio GJ: Second malignant neoplasms in survivors of Wilms’ tumor: A report from the National Wilms’ Tumor Study. J Natl Cancer Inst 80: 592, 1988.PubMedCrossRefGoogle Scholar
  58. 58.
    Mike V, Meadows AT, D’Angio GJ: Incidence of second malignant neoplasms in children: Results of an international study. Lancet 2: 1326, 1982.PubMedCrossRefGoogle Scholar
  59. 59.
    Li FP, Yan JC, Sallan S: Second neoplasms after Wilms’ tumor in childhood. J Natl Cancer Inst 71: 1205, 1983.PubMedGoogle Scholar
  60. 60.
    Tucker MA, Meadows AT, Boice JD: Cancer risk following treatment of childhood cancer. In: Radiation Carcinogenesis: Epidemiology and Biological Significance, Boice JD, Fraumeni JF Jr. (eds). Raven Press, New York, 1984, p. 211.Google Scholar
  61. 61.
    Li FP, Gimbrere K, Gelber RD, Sallan SE, Flamant F, Green DM, Heyn RM, Meadows AT: Outcome of pregnancy in survivors of Wilms’ tumor. JAMA 257: 216, 1987.PubMedCrossRefGoogle Scholar
  62. 62.
    LI FP, Fine W, Jaffe N: Offspring of patients treated for cancer in childhood. J Natl Cancer Inst 62: 1193, 1979.PubMedGoogle Scholar
  63. 63.
    Knudson AG Jr, Strong LC: Mutation and cancer: A model for Wilms’ tumor of the kidney. J Natl Cancer Inst 48: 313, 1972.PubMedGoogle Scholar
  64. 64.
    Matsunaga E: Genetics of Wilms’ tumor. Hum Genet 57: 231, 1981.PubMedCrossRefGoogle Scholar
  65. 65.
    Pendergrass TW: Congenital anomalies in chidren with Wilms’ tumor. A new survey. Cancer 37: 403, 1976.PubMedCrossRefGoogle Scholar
  66. 66.
    Pastore G, Modesto C, Lemerle J, Tournade MF, Voute PA, Rey A, Vurgers JMV, Zucker JM, Gurger D, deKraker J, Delemarre JFM: Epidemiological features of Wilms’ tumor: Results of studies by the International Society of Paediatric Oncology (SIOP). Med Ped Oncol 16: 7, 1988.CrossRefGoogle Scholar
  67. 67.
    Ledlie EM, Mynors LS, Draper GJ, Gorbach PD: Natural history and treatment of Wilms’ tumor: An analysis of 335 cases occurring in England and Wales 1962–1966. Br Med J 4:195, 1970.PubMedCrossRefGoogle Scholar
  68. 68.
    Breslow NE, Beckwith JB: Epidemiological features of Wilms’ tumor: Results of the National Wilms’ tumor Study. J Natl Cancer Inst 68: 429, 1982.PubMedGoogle Scholar
  69. 69.
    Knudson AG, Jr: Genetics and the child cured of cancer. In: Status of the Curability of Childhood Cancer, van Eys J, Sullivan MP (eds). Raven Press, New York, 1980, p. 295.Google Scholar
  70. 70.
    Brodeur GM: Genetic and cytogenetic aspects of Wilms’ tumor. In: Wilms’ Tumor: Clinical and Biological Manifestations, Pochedly C, Baum ES (eds). Elsevier Science Publishing, New York, p. 125.Google Scholar
  71. 71.
    Miller RM, Fraumeni JF Jr, Manning MD: Association of Wilms’ tumor with aniridia, hemihypertrophy and other congenital malformations. N Engl J Med 270: 922, 1964.PubMedCrossRefGoogle Scholar
  72. 72.
    DiGeorge AM, Harley RD: The association of aniridia, Wilms’ tumor, and genital abnormalities. Arch Opthalmol 75: 796, 1966.Google Scholar
  73. 73.
    Maurer HS, Pendergrass TW, Borges W, et al.: The role of genetic factors in the etiology of Wilms’ tumor. Cancer 43: 205, 1979.PubMedCrossRefGoogle Scholar
  74. 74.
    Francke U, George DL, Brown MG, et al.: Gene dose effect: Intraband mapping of the LDH A locus using cells from four individuals with different interstitial deletions of lip. Cytogenet Cell Genet 19: 197, 1977.PubMedCrossRefGoogle Scholar
  75. 75.
    Riccardi VM, Sujansky E, Smith AC, Francke U: Chromosomal imbalance in the aniridia Wilms’ tumor association: lip interstitial deletion. Pediatrics 61: 604, 1978.PubMedGoogle Scholar
  76. 76.
    Riccardi VM, Hittner HM, Francke U, Yunis JJ, Ledbetter D, Borges W: The aniridia Wilms’ tumor association: The critical role of chromosome band llpl3. Cancer Genet Cytogenet 2: 131, 1980.CrossRefGoogle Scholar
  77. 77.
    Fearon ER, Vogelstein B, Feinberg AP: Somatic deletion and duplication of genes on chromosome 11 in Wilms’ tumours. Nature 309: 176, 1984.PubMedCrossRefGoogle Scholar
  78. 78.
    Koufos A, Hansen MF, Lampkin BC, Workman ML, Copeland NG, Jenkins NA, Cavenee WL: Loss of alleles at loci on human chromosome 11 during genesis of Wilms’ tumor. Nature 309: 170, 1984.PubMedCrossRefGoogle Scholar
  79. 79.
    Koufos A, Hansen MF, Copeland NG, Jenkins NA, Lampkin BC, Cavenee WK: Loss of heterozygosity in three embryonal tumours suggests a common pathogenetic mechanism. Nature 316: 330, 1985.PubMedCrossRefGoogle Scholar
  80. 80.
    Riccardi VM, Hittner HM, Strong LC, et al.: Wilms’ tumor with aniridia/iris dysplasia and apparently normal chromosomes. J Pediatr 100: 574, 1982.PubMedCrossRefGoogle Scholar
  81. 81.
    Weiacker P, Muller CR, Mayer A, et al.: Assignment of the gene coding for human catalase to the short arm of chromosome 11. Ann Genet 23: 73, 1980.Google Scholar
  82. 82.
    Junien L, Turleau C, deGrouchy J, et al.: Regional assignment of catalase (CAT) gene to band llpl3: Association with aniridia-Wilms’ tumor gonadoblastoma (WAGR) complex. Ann Genet 23: 165, 1980.PubMedGoogle Scholar
  83. 83.
    Ferrell RE, Riccardi VM: Catalase levels in patients with aniridia and/or Wilms’ tumor: Utility and limitations. Cytogenet Cell Genet 31: 121, 1981.CrossRefGoogle Scholar
  84. 84.
    Harper ME, Ulrich A, Saunders GF: Localization of the human insulin gene to the distal end of the short arm of chromosome 11. Proc Natl Acad Sci USa 78: 4458, 1981.PubMedCrossRefGoogle Scholar
  85. 85.
    Compton DA, Weil MM, Jones C, Riccardi VM, Strong LC Saunders GF: Long range physical map of the Wilms’ tumor aniridia region on human chromosome 11. Cell 55: 827, 1988.PubMedCrossRefGoogle Scholar

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© Kluwer Academic Publishers, Boston 1989

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  • Ellen Shapiro

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