CC BY-NC-ND 4.0 · South Asian J Cancer 2022; 11(03): 260-268
DOI: 10.1055/s-0042-1743414
Original Article
Pediatric Cancer

Profile and Clinical Outcome of Children with Wilms' Tumor treated at a Tertiary Care Centre, India

Pritanjali Singh
1   Department of Radiotherapy, All India Institute of Medical Sciences, Patna, India
,
1   Department of Radiotherapy, All India Institute of Medical Sciences, Patna, India
,
Bindey Kumar
2   Department of Pediatric Surgery, All India Institute of Medical Sciences, Patna, India
,
Prem Kumar
3   Department of Radiodiagnosis, All India Institute of Medical Sciences, Patna, India
,
Punam Prasad Bhadani
4   Department of Pathology, All India Institute of Medical Sciences, Patna, India
› Author Affiliations
Funding None.

Abstract

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Pritanjali Singh

Background Wilms' tumor (WT) is the most common kidney tumor of the pediatric age group. The outcome of WT has improved due to the evolution of the treatment approach. A prospective observational study was conducted at All India Institute of Medical Sciences (AIIMS), Patna, to analyze the clinical profile along with the response and outcome to neoadjuvant chemotherapy according to the International Society of Pediatric Oncology (SIOP) protocol.

Materials and Methods In total, 28 patients of WT visited the radiotherapy department from January 2015 to December 2019.

Results Gender distribution showed male preponderance with a median age at diagnosis was 31 months. The abdominal lump was the dominant clinical presentation. The median volume of tumor at diagnosis was 359.48 mL (52.67–1805.76). Radiological staging workup shows that stage I, II, III, IV, and V were 7.1%, 39.3%, 39.3%, 10.7%, and 3.6% respectively. Neoadjuvant chemotherapy (NACT) was received by all patients. Also, 71.4% of patients showed > 50% of tumor volume reduction, while 28.6% of patients showed < 50% of tumor mass reduction. There was a statistically significant decrease in the tumor volume reduction following neoadjuvant chemotherapy (p < 0.001). There was a statistically significant stage down (p = 0.018) of the disease. Bivariate correlation studies showed recurrence was correlating statistically significantly with age < 24 months (p = 0.049), locoregional lymph nodes (p = 0.008), histopathological subtypes (p < 0.001), stage of the disease (p = 0.003), and risk groups (p < 0.001). In addition, 25% of patients developed recurrence during the median follow-up of 25 months. The median disease-free survival (DFS) and overall survival (OS) were not reached. The mean DFS and OS were 48 and 59.13 months, respectively. One- and 3-year DFS were 100% and 64.1%, respectively. One- and 3-year OS were 100% and 75% respectively.

Conclusion Our study suggests that most of the patients presented at an advanced stage, thus rendering most of the cases difficult to undergo surgery at presentation. Neoadjuvant chemotherapy followed by surgery may be considered a well-balanced approach with a comparable response and survival outcomes.

Ethical Approval

The Institutional Research Committee (IRC) at the All India Institute of Medical Sciences, Patna has approved the study (Ref. number – IEC 20; dated 23/12/14).


Authors' Contributions

PS, DS, BK, PK, and PD participated in the acquisition of data and drafting the manuscript. All authors read and approved the final manuscript.




Publication History

Article published online:
22 March 2022

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  • References

  • 1 Breslow N, Olshan A, Beckwith JB, Green DM. Epidemiology of Wilms tumor. Med Pediatr Oncol 1993; 21 (03) 172-181
  • 2 Wani SQ, Khan T, Wani SY, Lone MM, Afroz F. Wilm's tumor-collaborative approach is needed to prevent tumor upstaging and radiotherapy delays: a single institutional study. Indian J Med Paediatr Oncol 2019; 40: 409-412
  • 3 Dome JS, Huff V. Wilms Tumor Predisposition. 2003 Dec 19 [Updated 2016 Oct 20]. In: Adam MP, Ardinger HH, Pagon RA. et al., eds. GeneReviews® [Internet]. Seattle (WA): University of Washington; Seattle; 1993–2020. Accessed February 11, 2021 from: https://www.ncbi.nlm.nih.gov/books/NBK1294/
  • 4 Ghafoor T, Bashir F, Ahmed S, Khalil S, Farah T. Predictors of treatment outcome of Wilms tumour in low-income country; single centre experience from Pakistan. J Pediatr Urol 2020; 16 (03) 375.e1-375.e7
  • 5 Bhatnagar S. Management of Wilms' tumor: NWTS vs SIOP. J Indian Assoc Pediatr Surg 2009; 14 (01) 6-14
  • 6 Ekenze SO, Nwangwu EI, Ezomike UO, Orji EI, Okafor OO. Continuing barriers to care of Wilms tumor in a low-income country. Pediatr Blood Cancer 2019; 66 (01) e27416 DOI: 10.1002/pbc.27416.
  • 7 Godzinski J. The current status of treatment of Wilms' tumor as per the SIOP trials. J Indian Assoc Pediatr Surg 2015; 20 (01) 16-20
  • 8 Prasad M, Vora T, Agarwala S. et al. Management of Wilms tumor: ICMR consensus document. Indian J Pediatr 2017; 84 (06) 437-445 DOI: 10.1007/s12098-017-2305-5.
  • 9 Eisenhauer EA, Therasse P, Bogaerts J. et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer 2009; 45 (02) 228-247
  • 10 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 (06) 547.e1-547.e7 DOI: 10.1016/j.jpurol.2018.05.020.
  • 11 Popov SD, Sebire NJ, Vujanic GM. Wilms' Tumour – Histology and Differential Diagnosis. In: van den Heuvel-Eibrink MM. ed. Wilms Tumor [Internet]. Brisbane (AU): Codon Publications; 2016. Mar. Table 3. [SIOP staging system]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK373364/table/tab1_3/ DOI: 10.15586/codon.wt.2016.ch1
  • 12 Guruprasad B, Rohan B, Kavitha S, Madhumathi DS, Lokanath D, Appaji L. Wilms' tumor: single centre retrospective study from South India. Indian J Surg Oncol 2013; 4 (03) 301-304
  • 13 Pritchard J, Imeson J, Barnes J. et al. Result of United Kingdom Children's Cancer Study group first Wilms' tumor study. J Clin Oncol 1995; 13 (01) 124-133
  • 14 D'Angio GJ, Breslow N, Beckwith JB. et al. Treatment of wilms' tumor. Results of third national wilms tumor study. Cancer 1989; 64 (02) 349-360
  • 15 Anil Kumar N, Bezawada Satish, Chaitanya SVenkata, Sree Gouri SR, Pulla Prasad. A retrospective study of Wilms tumour in our institute. International Journal of Contemporary Medical Research 2016; 3 (08) 2223-2225
  • 16 Reinhard H, Aliani S, Ruebe C, Stöckle M, Leuschner I, Graf N. Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study. J Clin Oncol 2004; 22 (22) 4500-4506 DOI: 10.1200/JCO.2004.12.099.
  • 17 Tröbs RB. Anatomical basis for Wilms tumor surgery. J Indian Assoc Pediatr Surg 2009; 14 (02) 50-54 DOI: 10.4103/0971-9261.55151.
  • 18 Rais F, Benhmidou N, Rais G. et al. Wilms tumor in childhood: single centre retrospective study from the National Institute of Oncology of Rabat and literature review. Pediatric Hematology Oncology Journal 2016; 1: 28-34
  • 19 Kieran K, Anderson JR, Dome JS. et al. Lymph node involvement in Wilms tumor: results from National Wilms Tumor Studies 4 and 5. J Pediatr Surg 2012; 47 (04) 700-706
  • 20 Israels T, Chagaluka G, Pidini D. et al. The efficacy and toxicity of SIOP preoperative chemotherapy in Malawian children with a Wilms tumour. Pediatr Blood Cancer 2012; 59 (04) 636-641
  • 21 Reddy RK, Kannaiyan L, Srirampur S, Irfan GM, Rao S. Neoadjuvant chemotherapy in neonatal Wilms' tumor. J NTR Univ Health Sci 2015; 4: 134-135
  • 22 D'Angio GJ, Evans A, Breslow N. et al. Thetreatment of Wilms' tumor: results of the second national wilms' tumor study. Cancer 1981; 47 (09) 2302-2311
  • 23 Zuppan CW, Beckwith JB, Luckey DW. Anaplasia in unilateral Wilms' tumor: a report from the national wilms' tumor study pathology center. Hum Pathol 1988; 19 (10) 1199-1209
  • 24 Breslow N, Churchill G, Beckwith JB. et al. Prognosis for Wilms' tumor patients with nonmetastatic disease at diagnosis–results of the second National Wilms' Tumor Study. J Clin Oncol 1985; 3 (04) 521-531
  • 25 Faria P, Beckwith JB, Mishra K. et al. Focal versus diffuse anaplasia in Wilms tumor–new definitions with prognostic significance: a report from the National Wilms Tumor Study Group. Am J Surg Pathol 1996; 20 (08) 909-920
  • 26 Breslow N, Sharples K, Beckwith JB. et al. Prognostic factors in nonmetastatic, favorable histology Wilms' tumor. Results of the Third National Wilms' Tumor Study. Cancer 1991; 68 (11) 2345-2353
  • 27 Weirich A, Ludwig R, Graf N. et al. Survival in nephroblastoma treated according to the trial and study SIOP-9/GPOH with respect to relapse and morbidity. Ann Oncol 2004; 15 (05) 808-820