Login Register Cart 0
Generic placeholder image

New Emirates Medical Journal

Editor-in-Chief
ISSN (Online): 0250-6882

Back
Journal Home About Journal Editorial Board Current Issue Volumes/Issues
Subscribe
Research Article

The Clinical Characteristics and Survival Profiles of Wilms Tumor in the United Arab Emirates: A Single-center Retrospective Analysis

Author(s): Abdulrahman Mohammad AlJassmi*, Asim Noor Rana, Hani Humad and Anjan Madasu

Volume 2, Issue 1, 2021

Published on: 11 January, 2021

Page: [84 - 90] Pages: 7

DOI: 10.2174/0250688203666210111153116

open_access

Abstract

Background: Wilms Tumor (WT) is the most common pediatric kidney tumor, yet the disease has variable clinical characteristics and prognostic outcomes across different populations.

Objective: This study aims to review the clinical characteristics and survival outcomes of children with WT who have received treatment at Dubai Hospital, UAE.

Methods: A retrospective study was conducted involving the medical records of confirmed WT children (aged < 14 years) who had received treatment between 2013 and 2018. Diagnosis should have been established based on the histopathological examination of operable tumors and needle biopsy for inoperable tumors. The Société Internationale d’Oncologie Pédiatrique (SIOP) WT 2001 protocol was used for patient management, and the UMBRELLA SIOP–RTSG 2016 Wilms tumor pathology and molecular biology protocol was used for histopathologic classification and clinical staging. The presenting sign and symptoms, the findings of diagnostic imaging techniques, histological grading, the received treatments, and follow-up outcomes were collected and analyzed. Kaplan-Meier survival analysis was used to carry out the survival analysis.

Results: Ten children were diagnosed with WT (median age of 3.40 years, 60% males). All patients presented with abdominal masses without prominent pain. Synchronous bilateral lesions were found at diagnosis in one patient and metastatic lesions in three patients. Four children were discharged against medical advice; five received treatment according to the SIOP WT 2001 regimen, while the remaining patient was managed in the United States according to the National Wilms Tumor Study Group (NWTSG) protocol. The overall 6-year and relapse-free survival rates were 90% and 80%, respectively.

Conclusion: The clinical characteristics and managemental outcomes of children presenting with WT are promising, possibly owing to adopting the SIOP protocol. Considering the small sample size, more large-scale, nation-wide studies are warranted.

Keywords: Wilms tumor, Kidney neoplasms, Chemotherapy, Hereditary, Survival, United Arab Emirates.

[1]
Wilms M. Die Mischgeschwülste der Niere. Leipzig, Germany: Verlag von Arthur Georgi 1899.
[2]
American Cancer Society - Cancer Statistics Center. Cancer Facts & Statistics USA 2018. Available from: https://cancerstatisticscenter.cancer.org/#!/childhood-cancer
[3]
Szychot E, Apps J, Pritchard-Jones K. Wilms’ tumor: biology, diagnosis and treatment. Transl Pediatr 2014; 3(1): 12-24.
[http://dx.doi.org/10.3978/j.issn.2224-4336.2014.01.09] [PMID: 26835318]
[4]
Davidoff AM. Wilms tumor. Adv Pediatr 2012; 59(1): 247-67.
[http://dx.doi.org/10.1016/j.yapd.2012.04.001] [PMID: 22789581]
[5]
Dumba M, Jawad N, McHugh K. Neuroblastoma and nephroblastoma: a radiological review. Cancer Imaging 2015; 15: 5-5.
[http://dx.doi.org/10.1186/s40644-015-0040-6] [PMID: 25889326]
[6]
Rabeh W, Akel S, Eid T, et al. Wilms tumor: Successes and challenges in management outside of cooperative clinical trials. Hematol Oncol Stem Cell Ther 2016; 9(1): 20-5.
[http://dx.doi.org/10.1016/j.hemonc.2015.12.006] [PMID: 26802622]
[7]
Wilde JCH, Lameris W, van Hasselt EH, Molyneux EM, Heij HA, Borgstein EG. Challenges and outcome of Wilms’ tumour management in a resource-constrained setting. Afr J Paediatr Surg 2010; 7(3): 159-62.
[http://dx.doi.org/10.4103/0189-6725.70416] [PMID: 20859020]
[8]
Dome JS, Graf N, Geller JI, et al. Advances in wilms tumor treatment and biology: Progress through international collaboration. J Clin Oncol 2015; 33(27): 2999-3007.
[http://dx.doi.org/10.1200/JCO.2015.62.1888] [PMID: 26304882]
[9]
Njuguna F, Martijn HA, Kuremu RT, et al. Wilms tumor treatment outcomes: perspectives from a low-income setting. J Glob Oncol 2016; 3(5): 555-62.
[http://dx.doi.org/10.1200/JGO.2016.005389] [PMID: 29094095]
[10]
Honeyman JN, Rich BS, McEvoy MP, et al. Factors associated with relapse and survival in Wilms tumor: a multivariate analysis. J Pediatr Surg 2012; 47(6): 1228-33.
[http://dx.doi.org/10.1016/j.jpedsurg.2012.03.030] [PMID: 22703798]
[11]
United Nation Development Programme. Human Development Data (1990-2017). 2018. Available from: http://hdr.undp.org/en/data
[12]
Nawaz A, Matta H, Jacobsz A, Shawis R, Mpofu C, Al-Salem A. Wilms’ Tumor: The Tawam Hospital experience. Ann Saudi Med 1999; 19(3): 257-60.
[http://dx.doi.org/10.5144/0256-4947.1999.257] [PMID: 17283469]
[13]
Nawaz A, Mpofu C, Shawis R, et al. Synchronous bilateral Wilms’ tumor. Pediatr Surg Int 1999; 15(1): 42-5.
[http://dx.doi.org/10.1007/s003830050509] [PMID: 9914354]
[14]
Tournade MF, Com-Nougué C, de Kraker J, et al. 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. J Clin Oncol 2001; 19(2): 488-500.
[http://dx.doi.org/10.1200/JCO.2001.19.2.488] [PMID: 11208843]
[15]
Vujanić GM, Gessler M, Ooms AHAG, et al. The UMBRELLA SIOP-RTSG 2016 Wilms tumour pathology and molecular biology protocol. Nat Rev Urol 2018; 15(11): 693-701.
[http://dx.doi.org/10.1038/s41585-018-0100-3] [PMID: 30310143]
[16]
Pshak TJ, Cho DS, Hayes KL, Vemulakonda VM. Correlation between CT-estimated tumor volume, pathologic tumor volume, and final pathologic specimen weight in children with Wilms’ tumor. J Pediatr Urol 2014; 10(1): 148-54.
[http://dx.doi.org/10.1016/j.jpurol.2013.08.001] [PMID: 24035637]
[17]
D’Angio GJ, Evans A, Breslow N, et al. The treatment of Wilms’ tumor: results of the Second National Wilms’ Tumor Study. Cancer 1981; 47(9): 2302-11.
[http://dx.doi.org/10.1002/1097-0142(19810501)47:9<2302::AID-CNCR2820470933>3.0.CO;2-K] [PMID: 6164480]
[18]
Forthofer RN, Lee ES, Hernandez M. Analysis of Survival Data. Biostatistics: A guide to design, analysis and discovery. 2nd ed. USA: Elsevier 2006; pp. 297-315.
[19]
Statistics and Research Center. Cancer Incidence in United Arab Emirates Annual Report of the UAE-National Cancer Registry UAE: Ministry of Health and Prevention 2015. Available from: https://www.mohap.gov.ae/Files/MOH_OpenData/1553/NATIONAL%20CANCER%20REGISTRY%20ANNUAL%20REPORT%20_%202015.pdf
[20]
Chan CC, To KF, Yuen HL, et al. A 20-year prospective study of Wilms tumor and other kidney tumors: A report from Hong Kong pediatric hematology and oncology study group. J Pediatr Hematol Oncol 2014; 36(6): 445-50.
[http://dx.doi.org/10.1097/MPH.0000000000000018] [PMID: 25050908]
[21]
Pritchard J, Imeson J, Barnes J, et al. Results of the United Kingdom Children’s Cancer Study Group first Wilms’ Tumor Study. J Clin Oncol 1995; 13(1): 124-33.
[http://dx.doi.org/10.1200/JCO.1995.13.1.124] [PMID: 7799012]
[22]
Yildiz I, Yüksel L, Ozkan A, et al. Multidisciplinary approach to Wilms’ tumor: 18 years of experience. Jpn J Clin Oncol 2000; 30(1): 17-20.
[http://dx.doi.org/10.1093/jjco/hyd001] [PMID: 10770563]
[23]
Naguib SF, El Haddad A, El Badawy SA, Zaghloul AS. Multidisciplinary approach to wilms’ tumor: a retrospective analytical study of 53 patients. J Egypt Natl Canc Inst 2008; 20(4): 410-23.
[PMID: 20571600]
[24]
Yao W, Li K, Xiao X, et al. Outcomes of Wilms’ tumor in eastern China: 10 years of experience at a single center. J Invest Surg 2012; 25(3): 181-5.
[http://dx.doi.org/10.3109/08941939.2011.615893] [PMID: 22583015]
[25]
Maher K. K H, S H. Wilms’ tumor in children: A single institution 10-year experience. Egypt J Hosp Med 2014; 55: 159-64.
[http://dx.doi.org/10.12816/0004501]
[26]
Al Mulhim I. Wilm’s Tumor in Children: A 10-year Experience from the Eastern Province of Saudi Arabia. Saudi J Kidney Dis Transpl 1997; 8(2): 123-6.
[PMID: 18417789]
[27]
Pastore G, Znaor A, Spreafico F, Graf N, Pritchard-Jones K, Steliarova-Foucher E. Malignant renal tumours incidence and survival in European children (1978-1997): Report from the Automated Childhood Cancer Information System project. Eur J Cancer 2006; 42(13): 2103-14.
[http://dx.doi.org/10.1016/j.ejca.2006.05.010] [PMID: 16919774]
[28]
Fawkner-Corbett DW, Howell L, Pizer BL, Dominici C, McDowell HP, Losty PD. Wilms’ tumor: Lessons and outcomes: A 25-year single center UK experience. Pediatr Hematol Oncol 2014; 31(5): 400-8.
[http://dx.doi.org/10.3109/08880018.2014.912709] [PMID: 24933082]
[29]
Doganis D, Panagopoulou P, Tragiannidis A, et al. Childhood nephroblastoma in Southern and Eastern Europe and the US: Incidence variations and temporal trends by human development index. Cancer Epidemiol 2018; 54: 75-81.
[http://dx.doi.org/10.1016/j.canep.2018.03.012] [PMID: 29655086]
[30]
Doganis D, Zborovskaya A, Trojanowski M, et al. Wilms tumour event-free and overall survival in Southern and Eastern Europe: Pooled analyses of clinical data from four childhood cancer registries (1999-2017). Eur J Cancer 2019; 115: 37-46.
[http://dx.doi.org/10.1016/j.ejca.2019.04.008] [PMID: 31082691]
[31]
Sen S, Kadamba P, Al-AbdulAaly M, Mammen KE, Ahmed S. Results of Wilms’ tumour management in two tertiary-care hospitals in Asia. Pediatr Surg Int 1998; 13(1): 42-4.
[http://dx.doi.org/10.1007/s003830050240] [PMID: 9391203]
[32]
John R, Kurian JJ, Sen S, et al. Clinical outcomes of children with Wilms tumor treated on a SIOP WT 2001 protocol in a tertiary care hospital in south India. J Pediatr Urol 2018; 14(6): 547.e1-7.
[http://dx.doi.org/10.1016/j.jpurol.2018.05.020] [PMID: 30017606]
[33]
de Kraker J, Delemarre JF, Lilien MR, Tournade MF. Misstaging in nephroblastoma. Causes and consequences. A report of the Sixth Nephroblastoma Trial and Study of the International Society of Paediatric Oncology. Eur J Pediatr Surg 1999; 9(3): 153-7.
[http://dx.doi.org/10.1055/s-2008-1072232] [PMID: 10427490]
[34]
Plesko I, Kramárová E, Stiller CA, Coebergh JW, Santaquilani M. Survival of children with Wilms’ tumour in Europe. Eur J Cancer 2001; 37(6): 736-43.
[http://dx.doi.org/10.1016/S0959-8049(01)00048-X] [PMID: 11311648]
[35]
Gatta G, Capocaccia R, Coleman MP, Ries LA, Berrino F. Childhood cancer survival in Europe and the United States. Cancer 2002; 95(8): 1767-72.
[http://dx.doi.org/10.1002/cncr.10833] [PMID: 12365026]
[36]
Pritchard-Jones K, Graf N, van Tinteren H, Craft A. Evidence for a delay in diagnosis of Wilms’ tumour in the UK compared with Germany: implications for primary care for children. Arch Dis Child 2016; 101(5): 417-20.
[http://dx.doi.org/10.1136/archdischild-2015-309212] [PMID: 26948824]

Rights & Permissions Print Cite
Article Metrics
8
1
© 2024 Bentham Science Publishers | Privacy Policy