Wilms' Tumor: Survival Analysis in Children Under 15 Treated at a Pediatric Oncology Unit of a Cancer Treatment Institution
Tumor de Wilms: análisis de supervivencia en niños menores de 15 años tratados en una unidad de oncología pediátrica de una institución para el tratamiento de cáncer
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At the National Institute of Cancerology (INC) in Colombia, the treatment of Wilms' tumor (WT) follows strategies from the National Wilms Tumor Study Group (NWTSG I to V). Objective: to describe the clinical characteristics, overall survival (OS) and event-free survival (EFS) of children under 15 years of age with WT, treated with a protocol based on NWTSG guidelines between 2008 and 2018. Methods: a retrospective observational study of a cohort of children under 15 years of age with a diagnosis of WT, treated according to NWTSG guidelines at the INC between January 1, 2008, and December 31, 2018. A descriptive analysis of demographic and clinical variables was performed. EFS and OS were calculated using the Kaplan-Meier method. Results: we identified 48 patients with a diagnosis of WT; the mean age was 3.95 years (±2.27), with a male-to-female ratio of 1.18. The majority (58%) presented with advanced-stage disease (III and IV). Unfavorable histology was observed in 16.7%, with a relapse rate of 31% and a mortality rate of 21%. The EFS and OS rates were 62% and 77.3%, respectively, at 10.4 years; the most common cause of mortality was relapse. Conclusion: at INC, most children with WT present with advanced-stage disease. Although survival rates achieved with multidisciplinary treatment are lower in middle-income countries compared to high-income countries, they are reasonably acceptable. Enhanced diagnostic testing is necessary to develop more precisely risk-adapted treatments.
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- Graf N, Bergeron C. Wilms and Other Renal Tumours. In: Caron HN, Biondi A, Boterberg T, Doz F. Editors. Cancer in Children. Seventh edition. New York: Oxford University Press; 2020. p. 231-240. DOI: https://doi.org/10.1093/med/9780198797210.003.0027
- Vujanic GM, Chang KTE, Tanaka Y, Tsuzuky T. Nephroblastoma. In: WHO classification of tumor editorial board, editors. Pediatric Tumours part B. Fifth edition. Lyon: International agency for research on cancer; 2022. p. 697-701.
- Globocan 2020 [Internet]. International Agency for Research on Cancer 2022. Disponible en: http://gco.iarc.fr
- Fernandez CV, Geller JI, Ehrlich PF, Van den Heuvel-Eibrink M, Graf N, et al. Renal Tumors. In: Pizzo PA, Poplack DC, editors. Principles and Practices of Pediatric Oncology. Eighth edition. Philadelphia: Wolters Kluwer; 2021. p. 673-689.
- Verma, Nishant MD; Kumar, Archana MD. Clinicoepidemiological Profile and Outcome of Children With Wilms Tumor in a Developing Country. Journal of Pediatric Hematology/Oncology [Internet]. 2016;38(7):p e213-e216. Disponible en: https://doi.org/10.1097/MPH.0000000000000603 DOI: https://doi.org/10.1097/MPH.0000000000000603
- Breslow N, Olshan A, Beckwith JB, Green DM. Epidemiology of Wilms tumor. Med Pediatr Oncol [Internet]. 1993;21(3):172-81. Disponible en: https://doi.org/10.1002/mpo.2950210305. DOI: https://doi.org/10.1002/mpo.2950210305
- Coppes MJ, Arnold M, Beckwith JB, Ritchey ML, D'Angio GJ, Green DM, Breslow NE. Factors affecting the risk of contralateral Wilms tumor development: a report from the National Wilms Tumor Study Group. Cancer [Internet]. 1999 Apr 1;85(7):1616-25. Disponible en: https://doi.org/10.1002/(sici)1097-0142(19990401)85:7<1616::aid-cncr26>3.0.co;2-4 DOI: https://doi.org/10.1002/(SICI)1097-0142(19990401)85:7<1616::AID-CNCR26>3.0.CO;2-4
- Spreafico F, Fernandez CV, Brok J, et al. Wilms tumour. Nat Rev Dis Primers [Internet]. 2021;7(1):75. Disponible en: https://doi.org/10.1038/s41572-021-00308-8 DOI: https://doi.org/10.1038/s41572-021-00308-8
- Pater L, Melchior P, Rübe C, Cooper BT, et al. Wilms tumor. Pediatr Blood Cancer [Internet]. 2021;68 Suppl 2:e28257. Disponible en: https://doi.org/10.1002/pbc.28257 DOI: https://doi.org/10.1002/pbc.28257
- 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 [Internet] 2017;265(4):835-840. Disponible en: https://doi.org/10.1097/SLA.0000000000001716 DOI: https://doi.org/10.1097/SLA.0000000000001716
- 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 [Internet] 2006;24(15):2352-8. Disponible en: https://doi.org/10.1200/JCO.2005.04.7852 DOI: https://doi.org/10.1200/JCO.2005.04.7852
- Fernandez CV, Mullen EA, Chi YY, et al. Outcome and Prognostic Factors in Stage III Favorable-Histology Wilms Tumor: A Report From the Children's Oncology Group Study AREN0532. J Clin Oncol [Internet]. 2018;36(3):254-261. Disponible en: https://doi.org/10.1200/JCO.2017.73.7999 Erratum in: J Clin Oncol. 2019;37(29):2710 DOI: https://doi.org/10.1200/JCO.2017.73.7999
- Dix DB, Seibel NL, Chi YY, et al. Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children's Oncology Group AREN0533 Study. J Clin Oncol [Internet]. 2018;36(16):1564-1570. Disponible en: https://doi.org/10.1200/JCO.2017.77.1931 DOI: https://doi.org/10.1200/JCO.2017.77.1931
- Bhutani N, Kajal P, Sharma U. Many faces of Wilms Tumor: Recent advances and future directions. Ann Med Surg (Lond) [Internet]. 2021;64:102202. Disponible en: https://doi.org/10.1016/j.amsu.2021.102202 DOI: https://doi.org/10.1016/j.amsu.2021.102202
- Maciaszek JL, Oak N, Nichols KE. Recent advances in Wilms' tumor predisposition. Hum Mol Genet [Internet]. 2020;29(R2):R138-R149. Disponible en: https://doi.org/10.1093/hmg/ddaa091 DOI: https://doi.org/10.1093/hmg/ddaa091
- Pastore G, Znaor A, Spreafico F, et al. Malignant renal tumours incidence and survival in European children (1978-1997): report from the Automated Childhood Cancer Information System project. Eur J Cancer [Internet] 2006;42(13):2103-14. Disponible en: https://doi.org/10.1016/j.ejca.2006.05.010 DOI: https://doi.org/10.1016/j.ejca.2006.05.010
- Charlton J, Irtan S, Bergeron C, Pritchard-Jones K. Bilateral Wilms tumour: a review of clinical and molecular features. Expert Rev Mol Med [Internet]. 2017;19:e8. Disponible en: https://doi.org/10.1017/erm.2017.8 DOI: https://doi.org/10.1017/erm.2017.8
- Perlman EJ. Pediatric renal tumors: practical updates for the pathologist. Pediatr Dev Pathol [Internet]. 2005;8(3):320-38. Disponible en: https://doi.org/10.1007/s10024-005-1156-7 DOI: https://doi.org/10.1007/s10024-005-1156-7
- Vujanić GM, Gessler M, Ooms A et al. The UMBRELLA SIOP-RTSG 2016 Wilms tumour pathology and molecular biology protocol. Nat Rev Urol. 2018;15(11):693-701 DOI: https://doi.org/10.1038/s41585-018-0100-3
- D'Angio GJ. The National Wilms Tumor Study: a 40 year perspective. Lifetime Data Anal [Internet]. 2007;13(4):463-70. Disponible en: https://doi.org/10.1007/s10985-007-9062-0 DOI: https://doi.org/10.1007/s10985-007-9062-0
- Stokes CL, Stokes WA, Kalapurakal JA, et al. Timing of Radiation Therapy in Pediatric Wilms Tumor: A Report From the National Cancer Database. Int J Radiat Oncol Biol Phys [Internet]. 2018 Jun 1;101(2):453-461. Disponible en: https://doi.org/10.1016/j.ijrobp.2018.01.110 DOI: https://doi.org/10.1016/j.ijrobp.2018.01.110
- Chagaluka G, Paintsil V, Renner L, Weijers J, Chitsike I, Borgstein E, Kamiza S, Afungchwi GM, Kouya F, Hesseling P, Molyneux E, Israels T.Pediatr Blood Cancer [Internet]. 2020;67(9):e28383. Disponible en: https://doi.org/10.1002/pbc.28383 DOI: https://doi.org/10.1002/pbc.28383
- Herrera-Toro NH, Peña-Aguirre L, Arango-Rave ME. Tumor de Wilms: experiencia de 12 años en dos hospitales de alto nivel en Medellín, Colombia. Iatreia [Internet]. 2019;32(2):82-91. Disponible en: https://doi.org/10.17533/udea.iatreia.13 DOI: https://doi.org/10.17533/udea.iatreia.13
- 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 [Internet]. 2016;34(26):3189-94. Disponible en: https://doi.org/10.1200/JCO.2015.66.1140 DOI: https://doi.org/10.1200/JCO.2015.66.1140
- Gadd S, Huff V, Skol AD, et al. Genetic changes associated with relapse in favorable histology Wilms tumor: A Children's Oncology Group AREN03B2 study. Cell Rep Med [Internet]. 2022;3(6):100644. Disponible en: https://doi.org/10.1016/j.xcrm.2022.100644 DOI: https://doi.org/10.1016/j.xcrm.2022.100644
- Grundy PE, Breslow NE, Li S, et al; National Wilms Tumor Study Group. 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 [Internet]. 2005;23(29):7312-21. Disponible en: https://doi.org/10.1200/JCO.2005.01.2799 DOI: https://doi.org/10.1200/JCO.2005.01.2799
- Dix DB, Fernandez CV, Chi YY, et al. AREN0532 and AREN0533 study committees. Augmentation of Therapy for Combined Loss of Heterozygosity 1p and 16q in Favorable Histology Wilms Tumor: A Children's Oncology Group AREN0532 and AREN0533 Study Report. J Clin Oncol [Internet]. 2019;37(30):2769-2777. Disponible en: https://doi.org/10.1200/JCO.18.01972 DOI: https://doi.org/10.1200/JCO.18.01972
- Weil BR, Murphy AJ, Liu Q, et al. Late Health Outcomes Among Survivors of Wilms Tumor Diagnosed Over Three Decades: A Report From the Childhood Cancer Survivor Study. J Clin Oncol [Internet]. 2023;41(14):2638-2650. Disponible en: https://doi.org/10.1200/JCO.22.02111 DOI: https://doi.org/10.1200/JCO.22.02111
