Update on Wilms tumor
DOI:
https://doi.org/10.12775/JEHS.2023.46.01.031Keywords
wilms tumor, renal tumor, nephroblastomaAbstract
Background: Wilms' tumor is the most common childhood renal neoplasm. Among the worst prognosis forms are the bilateral form. The symptom complex is non-specific for malignancy; the first symptoms may include hypertension. Hematuria occurs in 30% of patients. The incidence of distant metastases at the time of diagnosis in children with Wilms' tumor is estimated to be about 20%.The purpose of this article is to present a review of the literature on the standardization of diagnosis, the latest treatment standards and to assess the prognosis of Wilms' Tumor.
Matherial and methods: Using PubMed, SCOPUS and Web of Science databases, the authors reviewed the peer-reviewed international literature from 1980-2023 using the keywords: "Wilms tumor," "renal tumor," "nephroblastoma."
Discussion: Bilateral tumors have the worst prognosis - according to the National Wilms Tumor Study Group, long-term survival is achieved in only 12% to 56% of patients. The prognosis of Wilms' tumor is also dependent on the occurrence of recurrences - local as well as localized outside the renal tissue. A factor directly affecting long-term survival is recurrence, mainly observed within 24 months after the end of therapy. The limitations of conventional therapies, including surgery, chemotherapy and radiotherapy, in preventing recurrence in WT patients and their potential to cause long-term side effects.
Conclusions:
Bilateral Wilms tumor is associated with more aggressive therapy than unilateral disease.In single kidney disease, therapeutic management is aimed at radical removal of all tumor foci, whereas in bilateral tumor, the goal of any management should additionally be to try to preserve the best possible renal function.
Screening in children is important in cancer. General physicians should refer patients for additional imaging studies when there are any diagnostic doubts, as cancer is characterized by a long, asymptomatic development.
References
Gratias EJ, Dome JS, Jennings LJ, Chi YY, Tian J, Anderson J 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;34(26):3189–94.
Irtan S, Ehrlich PF, Pritchard-Jones K. Wilms tumor: "State-of-the-art" update. Semin Pediatr Surg. 2016;25(5):250–6.
Termuhlen AM, Tersak JM, Liu Q, Yasui Y, Stovall M, Weathers R, et al. Twenty-five year follow-up of childhood Wilms tumor: a report from the Childhood Cancer Survivor Study. Pediatr. Blood Cancer. 2011;57(7):1210–6.
Wong KF, Reulen RC, Winter DL, Guha J, Fidler MM, Kelly J, et al. Risk of Adverse Health and Social Outcomes Up to 50 Years After Wilms Tumor: The British Childhood Cancer Survivor Study. J. Clin. Oncol. 2016;34(15):1772–9.
Bernstein L, Linet M, Smith MA, Olshan AF. Cancer incidence and survival among children and adolescents. National Cancer Institute. SEER Program. Bethesda. NIH. 1999;99(4649):79–90.
Kramer S, Meadows AT, Jarrett P. Racial variation in incide nce of Wilms` tumor: relationship to congenital anomalies. Med. Pediatr. Oncol. 1984;12:401–405.
Aydın B, Akyüz C, Yalçın B, Ekinci S, Oğuz B, Akçören Z et al. Bilateral Wilms tumors: Treatment results from a single center. Turk. J. Pediatr. 2019;61(1):44-51.
Paya K, Horcher E, Lawrenz K, Rebhandl W, Zoubek A. Bilateral Wilms' tumor--surgical aspects. Eur. J. Pediatr. Surg. 2001;11(2):99-104.
Pendergrass TW. Congenital anomalies in children with Wilms' tumor: a new survey. Cancer. 1976;37(1):403-8.
Shuman C, Kalish JM, Weksberg R. Beckwith-Wiedemann Syndrome. Seattle (WA): University of Washington. Seattle. 2023.
Green DM. The diagnosis and management of Wilms' tumor. Pediatr. Clin. North. Am. 1985;32(3):735-54.
Vujanić GM, Sandstedt B, Harms D, Kelsey A, Leuschner I, de Kraker J. Nephroblastoma Scientific Committee. Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med. Pediatr. Oncol. 2002;38(2):79-82.
Granger J, Gidvani VK. Acquired factor VII deficiency associated with Wilms tumor. Pediatr. Blood. Cancer. 2009;52(3):394-5.
Servaes SE, Hoffer FA, Smith EA, Khanna G. Imaging of Wilms tumor: an update. Pediatr. Radiol. 2019;49(11):1441-1452.
Fogelström A, Caldeman C, Wester T, Löf Granström A, Mesas Burgos C. Prevalence of Beckwith Wiedemann Syndrome and Risk of Embryonal Tumors in Children Born with Omphalocele. J. Pediatr. Surg. 2023;31:0022-3468.
Ehrlich PF, Ferrer FA, Ritchey ML, Anderson JR, Green DM, Grundy PE et al. Hepatic metastasis at diagnosis in patients with Wilms tumor is not an independent adverse prognostic factor for stage IV Wilms tumor: a report from the Children's Oncology Group/National Wilms Tumor Study Group. Ann. Surg. 2009;250(4):642-8.
Li T, Wang H, Chen X, He L. Case report: The CT features of pediatric retroperitoneal extrarenal Wilms tumor: a report of two cases and literature review. Front Pediatr. 2023;11:1161603.
Liu B, Abraham N, Chitsike I, Sylvie CGL, Kambugu J, Stévy NMA et al. Enhancing information on stage at diagnosis for childhood cancer in Africa. Pediatr. Blood Cancer. 2023:30555.
Coppes MJ, de Kraker J, van Dijken PJ, Perry HJ, Delemarre JF, Tournade MF et al. Bilateral Wilms' tumor: long-term survival and some epidemiological features. J. Clin. Oncol. 1989;7(3):310-5.
Kim S, Chung DH. Pediatric solid malignancies: neuroblastoma and Wilms' tumor. Surg. Clin. North. Am. 2006;86(2):469-87.
National Wilms 'Tumor Study Committee. Wilms' tumor: status report. J. Clin. Oncol. 1991;9:877–887.
Zahir M, Alidousti A, Kajbafzadeh AM, Arshadi H, Kompani F, Hajivalizadeh S et al. Current status and future perspectives of wilms tumor treatment in Iran. Ann. Med. Surg. (Lond). 2023;85(5):1425-1429.
Godzinski J. The current status of treatment of Wilms' tumor as per the SIOP trials. J. Indian Assoc. Pediatr. Surg. 2015;20(1):16-20.
Israels T. Wilms tumor in Africa: challenges to cure. Pediatr. Blood Cancer. 2012;58(1):3-4.
Chui CH. Editorial: Recent advances in organ-sparing techniques in pediatric solid tumor surgery. Front Pediatr. 2023;11:1247287.
Sonn G, Shortliffe LM. Management of Wilms tumor: current standard of care. Nat. Clin. Pract. Urol. 2008;5(10):551-60.
Israels T. Wilms tumor in Africa: challenges to cure. Pediatr. Blood Cancer. 2012;58(1):3-4.
Saltzman AF. Cost NG, Romao RLP. Wilms Tumor. Urol. Clin. North Am. 2023;50(3):455-464.
Szavay P, Luithle T, Garf N, Furtwangler R, Fuchs J. Primary hepatic metastases in nephroblastoma - a report of the SIOP / GPOH study. J. Pediatr. Surg. 2006;41:168–172.
Varan A. Wilms' tumor in children: an overview. Nephron. Clin. Pract. 2008;108(2):83-90.
Breslow NE, Collins AJ, Ritchey ML, Grigoriev YA, Peterson SM, Green DM. 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;174(5):1972-5.
Rudin C. Pritchard J. Fernando ON. Duffy PG. Trompeter RS. Renal transplantation in the management of bilateral Wilms 'tumor (BWT) and of Denys – Drash syndrome (DDS). Nephrol. Dial. Transplant. 1998;13(6):1506–1510.
Ertan S, Ehrlich PF, Pritchard-Jones K. Wilms tumor: "State-of-the-art" update. Semin. Pediatr. Surg. 2016;25(5):250-256.
Brok J, Lopez-Yurda M, Tinteren HV, et al. Relapse of Wilms 'tumor and detection methods: a retrospective analysis of the 2001 Renal Tumor Study Group - International Society of Pediatric Oncology Wilms' Tumor Protocol Database. Lancet Oncol. 2018;19:1072–1081.
Grundy P, Breslow N, Green DM, Sharples K, Evans A, D'Angio GJ. Prognostic factors for children with recurrent Wilms' tumor: results from the Second and Third National Wilms' Tumor Study. J. Clin. Oncol. 1989;7(5):638-47.
Szavay P, Luithle T, Garf N, Furtwangler R, Fuchs J. Primary hepatic metastases in nephroblastoma - a report of the SIOP / GPOH study. J. Pediatr. Surg. 2006;41:168–172.
Varan A. Wilms' tumor in children: an overview. Nephron. Clin. Pract. 2008;108(2):83-90.
Sanatkar SA, Heidari A, Arya S, Ghasemi M, Rezaei N. The potential role of immunotherapy in Wilms' tumor: opportunities and challenges. Curr Pharm Des. 2023:21.
Jha SK, Brown C, Kang L, Diaz ES, Gwal K, Alvarez E et al. Update on the Role of Imaging in Staging of Common Pediatric Abdominal Tumors. Curr. Probl. Cancer. 2023;47(2):100969.
Chen SY, Lee WG, Laifman E, Mack SJ, Zhou S, Kim ES. A Single Center Experience With Bilateral Wilms Tumor. Am Surg. 2023;19:31348231175446.
Leslie SW, Sajjad H, Murphy PB. Wilms Tumor. In: StatPearls [Internet]. Treasure Island (FL): 2023.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2023 Klaudia Kister, Jakub Laskowski, Paulina Bronst, Magdalena Mazur, Aleksandra Małolepsza, Monika Zach-Źródlak, Julia Czechowska, Lidia Rosa, Natalia Rektor, Anna Szabrańska
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
The periodical offers access to content in the Open Access system under the Creative Commons Attribution-NonCommercial-ShareAlike 4.0
Stats
Number of views and downloads: 257
Number of citations: 0