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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

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
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
Intensive chemotherapy with stem cell support-experience in pediatric solid tumours.
Pinkerton CR
Bull Cancer 1995;82 Suppl 1:61s-65s. PMID: 7626856
Acute toxicities associated with radiation in the second National Wilms' Tumor Study.
Thomas PR, Tefft M, D'Angio GJ, Norkool P
J Clin Oncol 1988 Nov;6(11):1694-8. doi: 10.1200/JCO.1988.6.11.1694. PMID: 2846791

Diagnosis

Renal and Urinary Conditions: Kidney Cysts and Tumors.
Sexton SJ
FP Essent 2024 Aug;543:12-17. PMID: 39163010
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
Adult Wilms tumour.
Zdrojowy R, Sawicz-Birkowska K, Apoznański W, Patkowski D, Szydełko T, Pietras W, Dembowski J
Int Urol Nephrol 2011 Sep;43(3):691-6. Epub 2010 Nov 26 doi: 10.1007/s11255-010-9868-7. PMID: 21110097
Wilm's tumour with WAGR complex.
Mathur P, Khamesera HL, Pendse AK, Chittora R, Porewal KK
Indian J Cancer 1996 Sep;33(3):136-8. PMID: 9055487
Inflammatory pseudotumor of the liver masquerading as a metastasis in a child treated for nephroblastoma.
Newbould MJ, Kelsey A, Lendon M, Gururangan S
Med Pediatr Oncol 1992;20(2):172-5. doi: 10.1002/mpo.2950200216. PMID: 1310343

Therapy

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
Adult Wilms tumour.
Zdrojowy R, Sawicz-Birkowska K, Apoznański W, Patkowski D, Szydełko T, Pietras W, Dembowski J
Int Urol Nephrol 2011 Sep;43(3):691-6. Epub 2010 Nov 26 doi: 10.1007/s11255-010-9868-7. PMID: 21110097
Recurrent adult nephroblastoma. Long-term remission after surgery plus adjuvant high-dose chemotherapy, radiation therapy, and allogeneic bone marrow transplantation.
Hupperets PS, Havenith MG, Blijham GH
Cancer 1992 Jun 15;69(12):2990-2. doi: 10.1002/1097-0142(19920615)69:12<2990::aid-cncr2820691221>3.0.co;2-6. PMID: 1317250
Acute toxicities associated with radiation in the second National Wilms' Tumor Study.
Thomas PR, Tefft M, D'Angio GJ, Norkool P
J Clin Oncol 1988 Nov;6(11):1694-8. doi: 10.1200/JCO.1988.6.11.1694. PMID: 2846791

Prognosis

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
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
Intensive chemotherapy with stem cell support-experience in pediatric solid tumours.
Pinkerton CR
Bull Cancer 1995;82 Suppl 1:61s-65s. PMID: 7626856
Wilm's tumor with pulmonary metastases at diagnosis: the significance of primary chemotherapy. International Society of Pediatric Oncology Nephroblastoma Trial and Study Committee.
de Kraker J, Lemerle J, Voûte PA, Zucker JM, Tournade MF, Carli M
J Clin Oncol 1990 Jul;8(7):1187-90. doi: 10.1200/JCO.1990.8.7.1187. PMID: 2162911

Clinical prediction guides

TRMT6 gene rs236110 C > A polymorphism increases the risk of Wilms tumor.
Chang X, Zhu J, Hua RX, Deng C, Zhang J, Cheng J, Li S, Zhou H, He J, Wang H
Gene 2023 Oct 5;882:147646. Epub 2023 Jul 19 doi: 10.1016/j.gene.2023.147646. PMID: 37473973
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
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
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

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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