Os tumores de Wilms com histologia favorável estão associados a taxas bem altas de sobrevida global de 5 anos de >90%.[107]D'Angio GJ. The National Wilms Tumor Study: a 40 year perspective. Lifetime Data Anal. 2007 Dec;13(4):463-70.
http://www.ncbi.nlm.nih.gov/pubmed/18027087?tool=bestpractice.com
[16]National Cancer Institute. Statistics for cancers in children, adolescents, and young adults. Oct 2024 [internet publication].
https://nccrexplorer.ccdi.cancer.gov
[2]Espinoza AF, Onwuka E, Siegel DA, et al. Incidence and survival of children and adolescents with Wilms tumor, United States, 2001-2020. Cancer Med. 2025 Feb;14(3):e70598.
https://onlinelibrary.wiley.com/doi/10.1002/cam4.70598
http://www.ncbi.nlm.nih.gov/pubmed/39928531?tool=bestpractice.com
No entanto, há um aumento do risco de morbidade e mortalidade relacionadas ao tratamento 25 anos depois do diagnóstico.[130]Termuhlen AM, Tersak JM, Liu Q, et al. Twenty-five year follow-up of childhood Wilms tumor: a report from the Childhood Cancer Survivor Study. Pediatr Blood Cancer. 2011 Dec 15;57(7):1210-6.
http://www.ncbi.nlm.nih.gov/pubmed/21384541?tool=bestpractice.com
Os pacientes com peso do tumor <550 g e idade <2 anos, em particular, apresentam excelentes desfechos e podem receber apenas nefrectomia, seguida de vigilância por imagem (de acordo com a abordagem de tratamento do Children's Oncology Group [COG]). Esses pacientes têm uma taxa de cura próxima a 90% com cirurgia isolada e evitam o risco de efeitos adversos da quimioterapia adjuvante.[98]Fernandez CV, Perlman EJ, Mullen EA, et al. Clinical outcome and biological predictors of relapse after nephrectomy only for very low-risk Wilms tumor: a report from Children's Oncology Group AREN0532. Ann Surg. 2017 Apr;265(4):835-40.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5145762
http://www.ncbi.nlm.nih.gov/pubmed/27811504?tool=bestpractice.com
[131]Green DM. The treatment of stages I-IV favorable histology Wilms' tumor. J Clin Oncol. 2004 Apr 15;22(8):1366-72.
http://www.ncbi.nlm.nih.gov/pubmed/15084612?tool=bestpractice.com
[132]Breslow NE, Beckwith JB, Haase GM, et al. Radiation therapy for favorable histology Wilms tumor: prevention of flank recurrence did not improve survival on National Wilms Tumor Studies 3 and 4. Int J Radiat Oncol Biol Phys. 2006 May 1;65(1):203-9.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1483841
http://www.ncbi.nlm.nih.gov/pubmed/16542795?tool=bestpractice.com
[133]Shamberger RC, Anderson JR, Breslow NE, et al. Long-term outcomes for infants with very low risk Wilms tumor treated with surgery alone in National Wilms Tumor Study-5. Ann Surg. 2010 Mar;251(3):555-8.
http://www.ncbi.nlm.nih.gov/pubmed/20142733?tool=bestpractice.com
A presença de anaplasia, aneuploidia, doença metastática à distância (especialmente para o linfonodo e/ou metástase hepática e/ou trombo tumoral intravascular) e perda de heterozigosidade (LOH) em 1p e 16q, ou ganho do cromossomo 1q no perfil genético do tumor conferem prognóstico desfavorável.[32]Gratias EJ, Jennings LJ, Anderson JR, et al. Gain of 1q is associated with inferior event-free and overall survival in patients with favorable histology Wilms tumor: a report from the Children's Oncology Group. Cancer. 2013 Nov 1;119(21):3887-94.
http://www.ncbi.nlm.nih.gov/pubmed/23983061?tool=bestpractice.com
[33]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 Oct 10;23(29):7312-21.
http://www.ncbi.nlm.nih.gov/pubmed/16129848?tool=bestpractice.com
[58]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 Feb;46(2):163-70.
http://www.ncbi.nlm.nih.gov/pubmed/17099873?tool=bestpractice.com
[59]Gratias EJ, Dome JS, Jennings LJ, et al. Association of chromosome 1q gain with inferior survival in favorable-histology Wilms tumor: a report from the Children's Oncology Group. J Clin Oncol. 2016 Sep 10;34(26):3189-94.
https://ascopubs.org/doi/10.1200/JCO.2015.66.1140?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
http://www.ncbi.nlm.nih.gov/pubmed/27400937?tool=bestpractice.com
[79]Skotnicka-Klonowicz G, Rieske P, Bartkowiak J, et al. 16q heterozygosity loss in Wilms' tumour in children and its clinical importance. Eur J Surg Oncol. 2000 Feb;26(1):61-6.
http://www.ncbi.nlm.nih.gov/pubmed/10718182?tool=bestpractice.com
[107]D'Angio GJ. The National Wilms Tumor Study: a 40 year perspective. Lifetime Data Anal. 2007 Dec;13(4):463-70.
http://www.ncbi.nlm.nih.gov/pubmed/18027087?tool=bestpractice.com
[117]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 May 20;24(15):2352-8.
http://www.ncbi.nlm.nih.gov/pubmed/16710034?tool=bestpractice.com
[123]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 Feb;50(2):236-41.
http://www.ncbi.nlm.nih.gov/pubmed/17539021?tool=bestpractice.com
[134]Osterheld MC, Caron L, Meagher-Villemure K. Role of DNA content analysis and immunohistochemistry in the evaluation of the risk of unfavourable outcome in Wilms' tumours. Anticancer Res. 2008 Mar-Apr;28(2A):751-6.
http://www.ncbi.nlm.nih.gov/pubmed/18507016?tool=bestpractice.com
[135]Nakadate H, Yokomori K, Watanabe N, et al. Mutations/deletions of the WT1 gene, loss of heterozygosity on chromosome arms 11p and 11q, chromosome ploidy and histology in Wilms' tumors in Japan. Int J Cancer. 2001 Nov 1;94(3):396-400.
http://onlinelibrary.wiley.com/doi/10.1002/ijc.1475/full
http://www.ncbi.nlm.nih.gov/pubmed/11745420?tool=bestpractice.com
[136]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 Mar;45(5):819-26.
http://www.ncbi.nlm.nih.gov/pubmed/19231157?tool=bestpractice.com
[137]Chagtai T, Zill C, Dainese L, et al. Gain of 1q as a prognostic biomarker in Wilms tumors (WTs) treated with preoperative chemotherapy in the International Society of Paediatric Oncology (SIOP) WT 2001 trial: a SIOP renal tumours biology consortium study. J Clin Oncol. 2016 Sep 10;34(26):3195-203.
https://ascopubs.org/doi/10.1200/JCO.2015.66.0001?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
http://www.ncbi.nlm.nih.gov/pubmed/27432915?tool=bestpractice.com
O comprometimento de linfonodos e a presença de doença residual microscópica são extremamente preditivos de sobrevida livre de eventos e sobrevida global em pacientes com doença em estádio III e histologia favorável.[138]Ehrlich PF, Anderson JR, Ritchey ML, et al. Clinicopathologic findings predictive of relapse in children with stage III favorable-histology Wilms tumor. J Clin Oncol. 2013 Mar 20;31(9):1196-201.
http://www.ncbi.nlm.nih.gov/pubmed/23382471?tool=bestpractice.com
O prognóstico é considerado excelente em pacientes com doença em estádio IV quando a histologia é favorável, a doença metastática está isolada no fígado ou nos pulmões e as metástases respondem ao tratamento com quimioterapia pré-operatória.[139]Berger M, Fernandez-Pineda I, Cabello R, et al. The relationship between the site of metastases and outcome in children with stage IV Wilms tumor: data from 3 European pediatric cancer institutions. J Pediatr Hematol Oncol. 2013 Oct;35(7):518-24.
http://www.ncbi.nlm.nih.gov/pubmed/23588334?tool=bestpractice.com
Os desfechos mais desfavoráveis são relatados em pacientes com metástases extrapulmonares de tumor de Wilms com histologia favorável, em comparação àqueles com metástases apenas pulmonares.[106]Benedetti DJ, Varela CR, Renfro LA, et al. Treatment of children with favorable histology Wilms tumor with extrapulmonary metastases: a report from the COG studies AREN0533 and AREN03B2 and NWTSG study NWTS-5. Cancer. 2024 Mar 15;130(6):947-61.
https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.35099
http://www.ncbi.nlm.nih.gov/pubmed/37933882?tool=bestpractice.com
O prognóstico é inferior para pacientes com tumores de alto risco em estádio IV; as taxas globais de sobrevida em 5 anos são de 17%, para o tumor de Wilms do tipo blastematoso em estádio IV, e de 16%, para o tumor de Wilms anaplásico difuso em estádio IV.[140]Pasqualini C, Furtwängler R, van Tinteren H, et al. Outcome of patients with stage IV high-risk Wilms tumour treated according to the SIOP2001 protocol: a report of the SIOP Renal Tumour Study Group. Eur J Cancer. 2020 Mar;128:38-46.
http://www.ncbi.nlm.nih.gov/pubmed/32109849?tool=bestpractice.com
As taxas de recorrência são baixas.[107]D'Angio GJ. The National Wilms Tumor Study: a 40 year perspective. Lifetime Data Anal. 2007 Dec;13(4):463-70.
http://www.ncbi.nlm.nih.gov/pubmed/18027087?tool=bestpractice.com
Os fatores prognósticos adversos para pacientes com tumores recorrentes incluem tratamento prévio com doxorrubicina, recidivas que ocorrem <12 meses após o diagnóstico, e recidiva intra-abdominal após irradiação abdominal.[107]D'Angio GJ. The National Wilms Tumor Study: a 40 year perspective. Lifetime Data Anal. 2007 Dec;13(4):463-70.
http://www.ncbi.nlm.nih.gov/pubmed/18027087?tool=bestpractice.com
[121]Speafico F, Pritchard Jones K, Malogolowkin MH, et al. Treatment of relapsed Wilms tumors: lessons learned. Expert Rev Anticancer Ther. 2009 Dec;9(12):1807-15.
http://www.ncbi.nlm.nih.gov/pubmed/19954292?tool=bestpractice.com
[141]Grundy P, Breslow N, Green DM, et al. Prognostic factors for children with recurrent Wilms' tumor: results from the Second and Third National Wilms' Tumor Study. J Clin Oncol. 1989 May;7(5):638-47.
http://www.ncbi.nlm.nih.gov/pubmed/2540289?tool=bestpractice.com
O prognóstico para pacientes com recorrência é desfavorável.
Desfechos desfavoráveis são observados em pacientes com síndrome de Denys-Drash, pois a doença geralmente é bilateral e está associada a insuficiência renal progressiva.[63]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 Nov;174(5):1972-5.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1483840
http://www.ncbi.nlm.nih.gov/pubmed/16217371?tool=bestpractice.com
[101]Heppe RK, Koyle MA, Beckwith JB. Nephrogenic rests in Wilms tumor patients with the Drash syndrome. J Urol. 1991 Jun;145(6):1225-8.
http://www.ncbi.nlm.nih.gov/pubmed/1851891?tool=bestpractice.com
[142]Breslow NE, Takashima JR, Ritchey ML, et al. Renal failure in the Denys-Drash and Wilms tumor-aniridia syndromes. Cancer Res. 2000 Aug 1;60(15):4030-2.
http://cancerres.aacrjournals.org/cgi/content/full/60/15/4030
http://www.ncbi.nlm.nih.gov/pubmed/10945603?tool=bestpractice.com
No entanto, a sobrevida global melhora com transplante renal bem-sucedido.[143]Kist-van Holthe JE, Ho PL, Stablein D, et al. Outcome of renal transplantation for Wilms' tumor and Denys-Drash syndrome: a report of the North American Pediatric Renal Transplant Cooperative Study. Pediatr Transplant. 2005 Jun;9(3):305-10.
http://www.ncbi.nlm.nih.gov/pubmed/15910385?tool=bestpractice.com
Um estudo constatou que a etnia hispânica pode estar associada a um prognóstico mais desfavorável; no entanto, pesquisas adicionais sobre disparidades étnicas relacionadas ao tumor de Wilms são necessárias.[3]Lovvorn HN 3rd, Renfro LA, Benedetti DJ, et al. Race and ethnic group enrollment and outcomes for Wilms tumor: analysis of the current era children's oncology group study, AREN03B2. J Am Coll Surg. 2024 Apr 1;238(4):733-49.
http://www.ncbi.nlm.nih.gov/pubmed/38251681?tool=bestpractice.com
[144]Amirian ES. The role of Hispanic ethnicity in pediatric Wilms' tumor survival. Pediatr Hematol Oncol. 2013 May;30(4):317-27.
http://www.ncbi.nlm.nih.gov/pubmed/23484868?tool=bestpractice.com