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Stage IV Kidney Wilms Tumor

MedGen UID:
78871
•Concept ID:
C0278714
•
Neoplastic Process
Synonyms: Stage IV Kidney Nephroblastoma; Stage IV Renal Wilms Tumor; Stage IV Renal Wilms' Tumor

Definition

Wilms tumor that has spread to other more distant organs, such as the lungs, liver, bones, and brain, or to lymph nodes outside the abdomen (National Wilms Tumor Study Group Staging System) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVStage IV Kidney Wilms Tumor

Professional guidelines

PubMed

Evidence-based surgical guidelines for treating children with Wilms tumor in low-resource settings.
Abdelhafeez AH, Reljic T, Kumar A, Banu T, Cox S, Davidoff AM, Elgendy A, Ghandour K, Gerstle JT, Karpelowsky J, Kaste SC, Kechiche N, Esiashvili N, Nasir A, Ngongola A, Marollano J, Moreno AA, Muzira A, Parkes J, Saldaña LJ, Shalkow J, Vujanić GM, Velasquez T, Lakhoo K, Mukkada S, Abib S
Pediatr Blood Cancer 2022 Dec;69(12):e29906. Epub 2022 Aug 5 doi: 10.1002/pbc.29906. PMID: 35929184
Management of Wilms Tumor: ICMR Consensus Document.
Prasad M, Vora T, Agarwala S, Laskar S, Arora B, Bansal D, Kapoor G, Chinnaswamy G, Radhakrishnan V, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 Jun;84(6):437-445. Epub 2017 Apr 3 doi: 10.1007/s12098-017-2305-5. PMID: 28367612
Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration.
Dome JS, Graf N, Geller JI, Fernandez CV, Mullen EA, Spreafico F, Van den Heuvel-Eibrink M, Pritchard-Jones K
J Clin Oncol 2015 Sep 20;33(27):2999-3007. Epub 2015 Aug 24 doi: 10.1200/JCO.2015.62.1888. PMID: 26304882Free PMC Article

Recent clinical studies

Etiology

Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children's Oncology Group AREN0533 Study.
Dix DB, Seibel NL, Chi YY, Khanna G, Gratias E, Anderson JR, Mullen EA, Geller JI, Kalapurakal JA, Paulino AC, Perlman EJ, Ehrlich PF, Malogolowkin M, Gastier-Foster JM, Wagner E, Grundy PE, Fernandez CV, Dome JS
J Clin Oncol 2018 Jun 1;36(16):1564-1570. Epub 2018 Apr 16 doi: 10.1200/JCO.2017.77.1931. PMID: 29659330Free PMC Article
Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group.
Ehrlich P, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Ritchey ML, Dome JS
Ann Surg 2017 Sep;266(3):470-478. doi: 10.1097/SLA.0000000000002356. PMID: 28795993Free PMC Article
Management of Wilms Tumor: ICMR Consensus Document.
Prasad M, Vora T, Agarwala S, Laskar S, Arora B, Bansal D, Kapoor G, Chinnaswamy G, Radhakrishnan V, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 Jun;84(6):437-445. Epub 2017 Apr 3 doi: 10.1007/s12098-017-2305-5. PMID: 28367612
Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration.
Dome JS, Graf N, Geller JI, Fernandez CV, Mullen EA, Spreafico F, Van den Heuvel-Eibrink M, Pritchard-Jones K
J Clin Oncol 2015 Sep 20;33(27):2999-3007. Epub 2015 Aug 24 doi: 10.1200/JCO.2015.62.1888. PMID: 26304882Free PMC Article
Laparoscopic approach for Wilms tumor.
Cabezalí Barbancho D, Guerrero Ramos F, López Vázquez F, Aransay Bramtot A, Gómez Fraile A
Surg Laparosc Endosc Percutan Tech 2014 Feb;24(1):22-5. doi: 10.1097/SLE.0b013e31829cebf1. PMID: 24487153

Diagnosis

Circulating Tumor DNA as a Biomarker in Patients With Stage III and IV Wilms Tumor: Analysis From a Children's Oncology Group Trial, AREN0533.
Madanat-Harjuoja LM, Renfro LA, Klega K, Tornwall B, Thorner AR, Nag A, Dix D, Dome JS, Diller LR, Fernandez CV, Mullen EA, Crompton BD
J Clin Oncol 2022 Sep 10;40(26):3047-3056. Epub 2022 May 17 doi: 10.1200/JCO.22.00098. PMID: 35580298Free PMC Article
Long-term kidney function in children with Wilms tumour and constitutional WT1 pathogenic variant.
Falcone MP, Pritchard-Jones K, Brok J, Mifsud W, Williams RD, Nakata K, Tugnait S, Al-Saadi R, Side L, Anderson J, Duncan C, Marks SD, Bockenhauer D, Chowdhury T
Pediatr Nephrol 2022 Apr;37(4):821-832. Epub 2021 Oct 4 doi: 10.1007/s00467-021-05125-5. PMID: 34608521Free PMC Article
Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group.
Ehrlich P, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Ritchey ML, Dome JS
Ann Surg 2017 Sep;266(3):470-478. doi: 10.1097/SLA.0000000000002356. PMID: 28795993Free PMC Article
Management of Wilms Tumor: ICMR Consensus Document.
Prasad M, Vora T, Agarwala S, Laskar S, Arora B, Bansal D, Kapoor G, Chinnaswamy G, Radhakrishnan V, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 Jun;84(6):437-445. Epub 2017 Apr 3 doi: 10.1007/s12098-017-2305-5. PMID: 28367612
Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration.
Dome JS, Graf N, Geller JI, Fernandez CV, Mullen EA, Spreafico F, Van den Heuvel-Eibrink M, Pritchard-Jones K
J Clin Oncol 2015 Sep 20;33(27):2999-3007. Epub 2015 Aug 24 doi: 10.1200/JCO.2015.62.1888. PMID: 26304882Free PMC Article

Therapy

Outcomes of Wilms tumor therapy in Lilongwe, Malawi, 2016-2021: Successes and ongoing research priorities.
Holmes DM, Matatiyo A, Mpasa A, Huibers MHW, Manda G, Tomoka T, Mulenga M, Namazzi R, Mehta P, Zobeck M, Mzikamanda R, Chintagumpala M, Allen C, Nuchtern JG, Borgstein E, Aronson DC, Ozuah N, Nandi B, McAtee CL
Pediatr Blood Cancer 2023 May;70(5):e30242. Epub 2023 Feb 16 doi: 10.1002/pbc.30242. PMID: 36798020Free PMC Article
Circulating Tumor DNA as a Biomarker in Patients With Stage III and IV Wilms Tumor: Analysis From a Children's Oncology Group Trial, AREN0533.
Madanat-Harjuoja LM, Renfro LA, Klega K, Tornwall B, Thorner AR, Nag A, Dix D, Dome JS, Diller LR, Fernandez CV, Mullen EA, Crompton BD
J Clin Oncol 2022 Sep 10;40(26):3047-3056. Epub 2022 May 17 doi: 10.1200/JCO.22.00098. PMID: 35580298Free PMC Article
Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children's Oncology Group AREN0533 Study.
Dix DB, Seibel NL, Chi YY, Khanna G, Gratias E, Anderson JR, Mullen EA, Geller JI, Kalapurakal JA, Paulino AC, Perlman EJ, Ehrlich PF, Malogolowkin M, Gastier-Foster JM, Wagner E, Grundy PE, Fernandez CV, Dome JS
J Clin Oncol 2018 Jun 1;36(16):1564-1570. Epub 2018 Apr 16 doi: 10.1200/JCO.2017.77.1931. PMID: 29659330Free PMC Article
Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group.
Ehrlich P, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Ritchey ML, Dome JS
Ann Surg 2017 Sep;266(3):470-478. doi: 10.1097/SLA.0000000000002356. PMID: 28795993Free PMC Article
Preoperative therapy for intracaval and atrial extension of Wilms tumor.
Ritchey ML, Kelalis PP, Haase GM, Shochat SJ, Green DM, D'Angio G
Cancer 1993 Jun 15;71(12):4104-10. doi: 10.1002/1097-0142(19930615)71:12<4104::aid-cncr2820711249>3.0.co;2-7. PMID: 8389661

Prognosis

Circulating Tumor DNA as a Biomarker in Patients With Stage III and IV Wilms Tumor: Analysis From a Children's Oncology Group Trial, AREN0533.
Madanat-Harjuoja LM, Renfro LA, Klega K, Tornwall B, Thorner AR, Nag A, Dix D, Dome JS, Diller LR, Fernandez CV, Mullen EA, Crompton BD
J Clin Oncol 2022 Sep 10;40(26):3047-3056. Epub 2022 May 17 doi: 10.1200/JCO.22.00098. PMID: 35580298Free PMC Article
Botryoid Wilms tumor: a non-existent "entity" causing diagnostic and staging difficulties.
Vujanić GM, Schiavo Lena M, Sebire NJ
Virchows Arch 2019 Feb;474(2):227-234. Epub 2018 Dec 4 doi: 10.1007/s00428-018-2500-4. PMID: 30515564
Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children's Oncology Group AREN0533 Study.
Dix DB, Seibel NL, Chi YY, Khanna G, Gratias E, Anderson JR, Mullen EA, Geller JI, Kalapurakal JA, Paulino AC, Perlman EJ, Ehrlich PF, Malogolowkin M, Gastier-Foster JM, Wagner E, Grundy PE, Fernandez CV, Dome JS
J Clin Oncol 2018 Jun 1;36(16):1564-1570. Epub 2018 Apr 16 doi: 10.1200/JCO.2017.77.1931. PMID: 29659330Free PMC Article
Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group.
Ehrlich P, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Ritchey ML, Dome JS
Ann Surg 2017 Sep;266(3):470-478. doi: 10.1097/SLA.0000000000002356. PMID: 28795993Free PMC Article
Ifosfamide in pediatric malignancies.
Jürgens H, Treuner J, Winkler K, Göbel U
Semin Oncol 1989 Feb;16(1 Suppl 3):46-50. PMID: 2649985

Clinical prediction guides

Circulating Tumor DNA as a Biomarker in Patients With Stage III and IV Wilms Tumor: Analysis From a Children's Oncology Group Trial, AREN0533.
Madanat-Harjuoja LM, Renfro LA, Klega K, Tornwall B, Thorner AR, Nag A, Dix D, Dome JS, Diller LR, Fernandez CV, Mullen EA, Crompton BD
J Clin Oncol 2022 Sep 10;40(26):3047-3056. Epub 2022 May 17 doi: 10.1200/JCO.22.00098. PMID: 35580298Free PMC Article
Management and outcome of pediatric metastatic Wilms' tumor at the National Cancer Institute, Egypt.
Elayadi M, Magdy S, Khalil E, Zekri W
J Egypt Natl Canc Inst 2020 Apr 15;32(1):19. doi: 10.1186/s43046-020-00031-7. PMID: 32372204
Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children's Oncology Group AREN0533 Study.
Dix DB, Seibel NL, Chi YY, Khanna G, Gratias E, Anderson JR, Mullen EA, Geller JI, Kalapurakal JA, Paulino AC, Perlman EJ, Ehrlich PF, Malogolowkin M, Gastier-Foster JM, Wagner E, Grundy PE, Fernandez CV, Dome JS
J Clin Oncol 2018 Jun 1;36(16):1564-1570. Epub 2018 Apr 16 doi: 10.1200/JCO.2017.77.1931. PMID: 29659330Free PMC Article
Management of pediatric renal tumor: Past and future trials of the Japan Wilms Tumor Study Group.
Oue T, Fukuzawa M, Koshinaga T, Okita H, Nozaki M, Chin M, Kaneko Y, Tanaka Y, Haruta M, Tsuchiya K, Kuwajima S, Takimoto T
Pediatr Int 2015 Oct;57(5):828-31. doi: 10.1111/ped.12787. PMID: 26267611
Challenge of pediatric oncology in Africa.
Hadley LG, Rouma BS, Saad-Eldin Y
Semin Pediatr Surg 2012 May;21(2):136-41. doi: 10.1053/j.sempedsurg.2012.01.006. PMID: 22475119

Recent systematic reviews

Evidence-based surgical guidelines for treating children with Wilms tumor in low-resource settings.
Abdelhafeez AH, Reljic T, Kumar A, Banu T, Cox S, Davidoff AM, Elgendy A, Ghandour K, Gerstle JT, Karpelowsky J, Kaste SC, Kechiche N, Esiashvili N, Nasir A, Ngongola A, Marollano J, Moreno AA, Muzira A, Parkes J, Saldaña LJ, Shalkow J, Vujanić GM, Velasquez T, Lakhoo K, Mukkada S, Abib S
Pediatr Blood Cancer 2022 Dec;69(12):e29906. Epub 2022 Aug 5 doi: 10.1002/pbc.29906. PMID: 35929184
The role of liver resection in metastatic nephroblastoma: a systematic review and Meta-regression analysis.
Fuchs J, Murtha-Lemekhova A, Kessler M, Günther P, Hoffmann K
BMC Cancer 2022 Jan 18;22(1):76. doi: 10.1186/s12885-022-09182-3. PMID: 35038991Free PMC Article
Clinical features, treatment, and outcomes of bilateral Wilms' tumor: A systematic review and meta-analysis.
Han Q, Li K, Dong K, Xiao X, Yao W, Liu G
J Pediatr Surg 2018 Dec;53(12):2465-2469. Epub 2018 Sep 1 doi: 10.1016/j.jpedsurg.2018.08.022. PMID: 30274708

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