Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 2002 Sep;87(3):241–244. doi: 10.1136/adc.87.3.241

Controversies and advances in the management of Wilms' tumour

K Pritchard-Jones
PMCID: PMC1719208  PMID: 12193442

Full Text

The Full Text of this article is available as a PDF (113.9 KB).

Figure 1 .

Figure 1

Open in a new tab

Cumulative risk of renal failure in five subgroups of patients defined hierarchically by the presence of a specific congenital anomaly or syndrome or intralobar nephrogenic rests (ILNR), DDS, Denys–Drash syndrome; WAGR, Wilms' tumour/aniridia syndrome; GU anomaly only, isolated male hypospadias or cryptorchidism; unilateral, ILNR, unilateral disease with ILNR but without any listed syndrome or anomaly; unilateral, no ILNR, unilateral disease without ILNR and without any syndrome or anomaly. Reproduced by permission of Edward Arnold Limited. NWTSG data provided by Dr N Breslow.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Beckwith J. B. Children at increased risk for Wilms tumor: monitoring issues. J Pediatr. 1998 Mar;132(3 Pt 1):377–379. doi: 10.1016/s0022-3476(98)70001-0. [DOI] [PubMed] [Google Scholar]
  2. Beckwith J. B. Nephrogenic rests and the pathogenesis of Wilms tumor: developmental and clinical considerations. Am J Med Genet. 1998 Oct 2;79(4):268–273. doi: 10.1002/(sici)1096-8628(19981002)79:4<268::aid-ajmg7>3.0.co;2-i. [DOI] [PubMed] [Google Scholar]
  3. Bergeron C., Iliescu C., Thiesse P., Bouvier R., Dijoud F., Ranchere-Vince D., Basset T., Chappuis J. P., Buclon M., Frappaz D. Does nephroblastomatosis influence the natural history and relapse rate in Wilms' tumour? A single centre experience over 11 years. Eur J Cancer. 2001 Feb;37(3):385–391. doi: 10.1016/s0959-8049(00)00409-3. [DOI] [PubMed] [Google Scholar]
  4. Bliek J., Maas S. M., Ruijter J. M., Hennekam R. C., Alders M., Westerveld A., Mannens M. M. Increased tumour risk for BWS patients correlates with aberrant H19 and not KCNQ1OT1 methylation: occurrence of KCNQ1OT1 hypomethylation in familial cases of BWS. Hum Mol Genet. 2001 Mar 1;10(5):467–476. doi: 10.1093/hmg/10.5.467. [DOI] [PubMed] [Google Scholar]
  5. Breslow N. E., Takashima J. R., Ritchey M. L., Strong L. C., Green D. M. Renal failure in the Denys-Drash and Wilms' tumor-aniridia syndromes. Cancer Res. 2000 Aug 1;60(15):4030–4032. [PubMed] [Google Scholar]
  6. Choyke P. L., Siegel M. J., Craft A. W., Green D. M., DeBaun M. R. Screening for Wilms tumor in children with Beckwith-Wiedemann syndrome or idiopathic hemihypertrophy. Med Pediatr Oncol. 1999 Mar;32(3):196–200. doi: 10.1002/(sici)1096-911x(199903)32:3<196::aid-mpo6>3.0.co;2-9. [DOI] [PubMed] [Google Scholar]
  7. Coppes M. J., Arnold M., Beckwith J. B., Ritchey M. L., D'Angio G. J., Green D. M., Breslow N. E. Factors affecting the risk of contralateral Wilms tumor development: a report from the National Wilms Tumor Study Group. Cancer. 1999 Apr 1;85(7):1616–1625. doi: 10.1002/(sici)1097-0142(19990401)85:7<1616::aid-cncr26>3.0.co;2-4. [DOI] [PubMed] [Google Scholar]
  8. Crolla J. A., Cawdery J. E., Oley C. A., Young I. D., Gray J., Fantes J., van Heyningen V. A FISH approach to defining the extent and possible clinical significance of deletions at the WAGR locus. J Med Genet. 1997 Mar;34(3):207–212. doi: 10.1136/jmg.34.3.207. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. DeBaun M. R., Siegel M. J., Choyke P. L. Nephromegaly in infancy and early childhood: a risk factor for Wilms tumor in Beckwith-Wiedemann syndrome. J Pediatr. 1998 Mar;132(3 Pt 1):401–404. doi: 10.1016/s0022-3476(98)70009-5. [DOI] [PubMed] [Google Scholar]
  10. Diller L., Ghahremani M., Morgan J., Grundy P., Reeves C., Breslow N., Green D., Neuberg D., Pelletier J., Li F. P. Constitutional WT1 mutations in Wilms' tumor patients. J Clin Oncol. 1998 Nov;16(11):3634–3640. doi: 10.1200/JCO.1998.16.11.3634. [DOI] [PubMed] [Google Scholar]
  11. Green D. M., Breslow N. E., Beckwith J. B., Finklestein J. Z., Grundy P. E., Thomas P. R., Kim T., Shochat S. J., Haase G. M., Ritchey M. L. 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 Jan;16(1):237–245. doi: 10.1200/JCO.1998.16.1.237. [DOI] [PubMed] [Google Scholar]
  12. Green D. M., Breslow N. E., Beckwith J. B., Finklestein J. Z., Grundy P., Thomas P. R., Kim T., Shochat S., Haase G., Ritchey M. Effect of duration of treatment on treatment outcome and cost of treatment for Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol. 1998 Dec;16(12):3744–3751. doi: 10.1200/JCO.1998.16.12.3744. [DOI] [PubMed] [Google Scholar]
  13. Green D. M., Breslow N. E., Beckwith J. B., Norkool P. Screening of children with hemihypertrophy, aniridia, and Beckwith-Wiedemann syndrome in patients with Wilms tumor: a report from the National Wilms Tumor Study. Med Pediatr Oncol. 1993;21(3):188–192. doi: 10.1002/mpo.2950210307. [DOI] [PubMed] [Google Scholar]
  14. Hastie N. D. The genetics of Wilms' tumor--a case of disrupted development. Annu Rev Genet. 1994;28:523–558. doi: 10.1146/annurev.ge.28.120194.002515. [DOI] [PubMed] [Google Scholar]
  15. Hoyme H. E., Seaver L. H., Jones K. L., Procopio F., Crooks W., Feingold M. Isolated hemihyperplasia (hemihypertrophy): report of a prospective multicenter study of the incidence of neoplasia and review. Am J Med Genet. 1998 Oct 2;79(4):274–278. [PubMed] [Google Scholar]
  16. Koziell A., Charmandari E., Hindmarsh P. C., Rees L., Scambler P., Brook C. G. Frasier syndrome, part of the Denys Drash continuum or simply a WT1 gene associated disorder of intersex and nephropathy? Clin Endocrinol (Oxf) 2000 Apr;52(4):519–524. doi: 10.1046/j.1365-2265.2000.00980.x. [DOI] [PubMed] [Google Scholar]
  17. Mitchell C., Jones P. M., Kelsey A., Vujanic G. M., Marsden B., Shannon R., Gornall P., Owens C., Taylor R., Imeson J. The treatment of Wilms' tumour: results of the United Kingdom Children's cancer study group (UKCCSG) second Wilms' tumour study. Br J Cancer. 2000 Sep;83(5):602–608. doi: 10.1054/bjoc.2000.1338. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Pelletier J., Bruening W., Kashtan C. E., Mauer S. M., Manivel J. C., Striegel J. E., Houghton D. C., Junien C., Habib R., Fouser L. Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome. Cell. 1991 Oct 18;67(2):437–447. doi: 10.1016/0092-8674(91)90194-4. [DOI] [PubMed] [Google Scholar]
  19. Pritchard-Jones K. Molecular genetic pathways to Wilms tumor. Crit Rev Oncog. 1997;8(1):1–27. doi: 10.1615/critrevoncog.v8.i1.10. [DOI] [PubMed] [Google Scholar]
  20. Rahman N., Arbour L., Houlston R., Bonaïti-Pellié C., Abidi F., Tranchemontagne J., Ford D., Narod S., Pritchard-Jones K., Foulkes W. D. Penetrance of mutations in the familial Wilms tumor gene FWT1. J Natl Cancer Inst. 2000 Apr 19;92(8):650–652. doi: 10.1093/jnci/92.8.650. [DOI] [PubMed] [Google Scholar]
  21. Rahman N., Arbour L., Tonin P., Renshaw J., Pelletier J., Baruchel S., Pritchard-Jones K., Stratton M. R., Narod S. A. Evidence for a familial Wilms' tumour gene (FWT1) on chromosome 17q12-q21. Nat Genet. 1996 Aug;13(4):461–463. doi: 10.1038/ng0896-461. [DOI] [PubMed] [Google Scholar]
  22. Rapley E. A., Barfoot R., Bonaïti-Pellié C., Chompret A., Foulkes W., Perusinghe N., Reeve A., Royer-Pokora B., Schumacher V., Shelling A. Evidence for susceptibility genes to familial Wilms tumour in addition to WT1, FWT1 and FWT2. Br J Cancer. 2000 Jul;83(2):177–183. doi: 10.1054/bjoc.2000.1283. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Schumacher V., Schneider S., Figge A., Wildhardt G., Harms D., Schmidt D., Weirich A., Ludwig R., Royer-Pokora B. Correlation of germ-line mutations and two-hit inactivation of the WT1 gene with Wilms tumors of stromal-predominant histology. Proc Natl Acad Sci U S A. 1997 Apr 15;94(8):3972–3977. doi: 10.1073/pnas.94.8.3972. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Schumacher V., Schärer K., Wühl E., Altrogge H., Bonzel K. E., Guschmann M., Neuhaus T. J., Pollastro R. M., Kuwertz-Bröking E., Bulla M. Spectrum of early onset nephrotic syndrome associated with WT1 missense mutations. Kidney Int. 1998 Jun;53(6):1594–1600. doi: 10.1046/j.1523-1755.1998.00948.x. [DOI] [PubMed] [Google Scholar]
  25. Shamberger R. C., Guthrie K. A., Ritchey M. L., Haase G. M., Takashima J., Beckwith J. B., D'Angio G. J., Green D. M., Breslow N. E. Surgery-related factors and local recurrence of Wilms tumor in National Wilms Tumor Study 4. Ann Surg. 1999 Feb;229(2):292–297. doi: 10.1097/00000658-199902000-00019. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Tournade M. F., Com-Nougué C., de Kraker J., Ludwig R., Rey A., Burgers J. M., Sandstedt B., Godzinski J., Carli M., Potter R. 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 Jan 15;19(2):488–500. doi: 10.1200/JCO.2001.19.2.488. [DOI] [PubMed] [Google Scholar]
  27. Veugelers M., Cat B. D., Muyldermans S. Y., Reekmans G., Delande N., Frints S., Legius E., Fryns J. P., Schrander-Stumpel C., Weidle B. Mutational analysis of the GPC3/GPC4 glypican gene cluster on Xq26 in patients with Simpson-Golabi-Behmel syndrome: identification of loss-of-function mutations in the GPC3 gene. Hum Mol Genet. 2000 May 22;9(9):1321–1328. doi: 10.1093/hmg/9.9.1321. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES