Editing Wilms' tumor (section)

==Treatment and prognosis==
The overall [[5-year survival]] is estimated to be approximately 90%,<ref>{{cite journal | vauthors = Stewénius Y, Jin Y, Øra I, de Kraker J, Bras J, Frigyesi A, Alumets J, Sandstedt B, Meeker AK, Gisselsson D | title = Defective chromosome segregation and telomere dysfunction in aggressive Wilms' tumors | journal = Clinical Cancer Research | volume = 13 | issue = 22 Pt 1 | pages = 6593–602 | date = November 2007 | pmid = 18006759 | doi = 10.1158/1078-0432.CCR-07-1081 | doi-access =  | s2cid = 17036977 }}</ref><ref>{{cite journal | vauthors = Tournade MF, Com-Nougué C, de Kraker J, Ludwig R, Rey A, Burgers JM, Sandstedt B, Godzinski J, Carli M, Potter R, Zucker JM | title = 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 | journal = Journal of Clinical Oncology | volume = 19 | issue = 2 | pages = 488–500 | date = January 2001 | pmid = 11208843 | doi = 10.1200/jco.2001.19.2.488 | author12 = International Society of Pediatric Oncology Nephroblastoma Trial Study Committee }}</ref> but for individuals the prognosis is highly dependent on individual [[#Staging|staging and treatment]]. Early removal tends to promote positive outcomes.

Tumor-specific loss-of-heterozygosity (LOH) for chromosomes 1p and 16q identifies a subset of Wilms' tumor patients who have a significantly increased risk of relapse and death. LOH for these chromosomal regions can now be used as an independent prognostic factor together with disease stage to target intensity of treatment to risk of treatment failure.<ref>{{cite journal | vauthors = Messahel B, Williams R, Ridolfi A, A'hern R, Warren W, Tinworth L, Hobson R, Al-Saadi R, Whyman G, Brundler MA, Kelsey A, Sebire N, Jones C, Vujanic G, Pritchard-Jones K | title = 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 | journal = European Journal of Cancer | volume = 45 | issue = 5 | pages = 819–26 | date = March 2009 | pmid = 19231157 | doi = 10.1016/j.ejca.2009.01.005 | author16 = Children's Cancer Leukaemia Group (CCLG) }}</ref><ref>{{cite journal |vauthors=Grundy PE, [[Breslow NE]], Li S, Perlman E, Beckwith JB, [[Ritchey ML]], Shamberger RC, Haase GM, D'Angio GJ, Donaldson M, Coppes MJ, Malogolowkin M, Shearer P, Thomas PR, Macklis R, Tomlinson G, Huff V, Green DM |date=October 2005 |title=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 |journal=Journal of Clinical Oncology |volume=23 |issue=29 |pages=7312–21 |doi=10.1200/JCO.2005.01.2799 |pmid=16129848 |doi-access=free |author19=National Wilms Tumor Study Group}}</ref> Genome-wide copy number and LOH status can be assessed with [[Virtual Karyotype|virtual karyotyping]] of tumor cells (fresh or paraffin-embedded).{{citation needed|date=April 2021}}

Statistics may sometimes show more favorable outcomes for more aggressive stages than for less aggressive stages, which may be caused by more aggressive treatment and/or [[random variability]] in the study groups. Also, a stage V tumor is not necessarily worse than, but nevertheless comparable in prognosis to a stage IV tumor.{{citation needed|date=April 2021}}
{|class="wikitable"
![[Cancer staging|Stage]]<ref name=NCI-Unless>Unless otherwise specified in boxes, then reference is: [http://www.cancer.gov/cancertopics/pdq/treatment/wilms/HealthProfessional/Page5 Treatment of Wilms Tumor] at [[National Cancer Institute]]. Last Modified: 03/29/2012</ref>!! [[Histopathology]]<ref name=NCI-Unless/> !! 4 Year [[relapse-free survival]] (RFS) or [[event-free survival]] (EFS)<ref name=NCI-Unless/> !! 4 Year [[overall survival]] (OS)<ref name=NCI-Unless/> !! Treatment<ref name=NCI-Unless/>
|-
!rowspan=3| I<ref name=NCI-Unless/>
| Favorable histology in children younger than 24 months or tumor weight less than 550g || 85% || 98% || Surgery only (should be done only within the context of a clinical trial)
|-
| Favorable histology in children older than 24 months or tumor weight more than 550g || 94% RFS || 98% || [[Nephrectomy]] + lymph node sampling followed by regimen [[EE-4A]]
|-
| Diffuse [[anaplastic]] || 68% EFS || 80% || Nephrectomy + lymph node sampling followed by regimen [[EE-4A]] and [[radiotherapy]]
|-
!rowspan=3| II<ref name=NCI-Unless/>
| Favorable histology || 86% RFS || 98% || Nephrectomy + lymph node sampling followed by regimen EE-4A
|-
| Focal anaplastic || 80% EFS || 80% || Nephrectomy + lymph node sampling followed by abdominal radiotherapy and regimen [[DD-4A]]
|-
| Diffuse anaplastic || 83% EFS || 82% || Nephrectomy + lymph node sampling followed by abdominal radiotherapy and [[regimen I]]
|-
!rowspan=5| III<ref name=NCI-Unless/>
| Favorable histology || 87% RFS || 94% || Nephrectomy + lymph node sampling followed by abdominal radiotherapy and regimen [[DD-4A]]
|-
| Focal anaplastic || 88% RFS || 100% (8 people in study) || Nephrectomy + lymph node sampling followed by abdominal radiotherapy and regimen DD-4A
|-
| Focal anaplastic (preoperative treatment) || 71% RFS || 71% || Preoperative treatment with regimen DD-4A followed by nephrectomy + lymph node sampling and abdominal radiotherapy
|-
| Diffuse anaplastic || 46% EFS || 53% || Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling and abdominal radiotherapy
|-
| Diffuse anaplastic || 65% EFS || 67% || Immediate nephrectomy + lymph node sampling followed by abdominal radiotherapy and regimen I
|-
!rowspan=4| IV<ref name=NCI-Unless/>
| Favorable histology || 76% RFS || 86% || Nephrectomy + lymph node sampling, followed by abdominal radiotherapy, bilateral pulmonary radiotherapy, and regimen DD-4A
|-
| Focal anaplastic || 61% EFS || 72% || Nephrectomy + lymph node sampling, followed by abdominal radiotherapy, bilateral pulmonary radiotherapy, and regimen DD-4A
|-
| Diffuse anaplastic || 33% EFS || 33% || Immediate nephrectomy + lymph node sampling followed by abdominal radiotherapy, whole-lung radiotherapy, and regimen I
|-
| Diffuse anaplastic (preoperative treatment) || 31% EFS || 44% || Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling followed by abdominal radiotherapy, whole-lung radiotherapy
|-
!rowspan=4| Bilateral (V)<ref name=NCI-Unless/>
| Overall || 61% EFS || 80% ||
|-
| Favorable histology || 65% || 87% || Preoperative treatment with regimen [[DD-4A]], followed by nephron sparing surgery or nephrecomy, staging of tumors, and chemotherapy and/or radiotherapy based on pathology and staging
|-
| Focal anaplastic || 76% || 88% || Preoperative treatment with regimen [[DD-4A]], followed by nephron sparing surgery or nephrecomy, staging of tumors, and chemotherapy and/or radiotherapy based on pathology and staging
|-
| Diffuse anaplastic || 25% || 42% || Preoperative treatment with regimen [[DD-4A]], followed by nephron sparing surgery or nephrecomy, staging of tumors, and chemotherapy and/or radiotherapy based on pathology and staging
|}

In case of relapse of Wilms' tumor, the 4-year survival rate for children with a standard-risk has been estimated to be 80%.<ref name="SpreaficoPritchard Jones2014">{{cite journal | vauthors = Spreafico F, Pritchard Jones K, Malogolowkin MH, Bergeron C, Hale J, de Kraker J, Dallorso S, Acha T, de Camargo B, Dome JS, Graf N | s2cid = 207212698 | title = Treatment of relapsed Wilms tumors: lessons learned | journal = Expert Review of Anticancer Therapy | volume = 9 | issue = 12 | pages = 1807–15 | date = December 2009 | pmid = 19954292 | doi = 10.1586/era.09.159 }}</ref>