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Protocol-based Management of Nephroblastoma: Five-year Outcomes with SIOP-RTSG Guidelines in an Indian Cohort
Nishant Agarwal1, Basant Kumar1, Ankur Mandelia1
1Department of Pediatric Surgery Superspecialities, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Insights
Pediatric Wilms' tumor (WT) survival in India is below 80% due to late diagnosis. Adhering to SIOP protocols improved outcomes, achieving 96% 1-year survival in a North Indian center.
Area of Science:
- Pediatric Oncology
- Nephrology
- Global Health
Background:
- Wilms' tumor (WT) is the most common childhood kidney cancer.
- High cure rates (>90%) are seen in developed nations.
- Low- and middle-income countries (LMICs) face challenges like late presentation and limited access to care, resulting in survival rates below 80%.
Purpose of the Study:
- To assess demographic characteristics of pediatric nephroblastoma patients.
- To evaluate risk categorization and survival outcomes.
- To analyze WT management at a tertiary care center in North India.
Main Methods:
- Retrospective analysis of 28 pediatric WT patients (Jan 2020 - Dec 2024).
- Data collection included demographics, clinical presentation, imaging, surgery, staging, and histopathology.
- Management followed the UMBRELLA SIOP-RTSG 2016 protocol.
Main Results:
- Median age was 27 months; male-to-female ratio was 2.5:1.
- Abdominal mass (93%) and hypertension (21%) were common presentations.
- 1-year overall survival (OS) and event-free survival (EFS) were 96% and 87%; 3-year OS and EFS were 85% and 82%.
Conclusions:
- Risk-adapted SIOP protocol adherence yielded favorable outcomes despite resource limitations.
- Complex cases like vascular thrombus and bilateral tumors showed good survival.
- Early diagnosis, chemotherapy, and surgical expertise are crucial for improving outcomes in LMICs.
Background:
Nephroblastoma or Wilms' tumor (WT) is the most common malignant renal tumor in children, with high cure rates (>90%) in developed countries. However, in low- and middle-income countries (LMICs) such as India, late presentation, limited access to care, and non-uniform adherence to protocols persist leads overall survival (OS) below 80%.The study aims to assess the demographic characteristics, risk categorization, and survival outcomes among children having nephroblastoma at our tertiary care center in North India.
Materials And Methods:
This retrospective study analyzed children diagnosed with WT at our center from January 2020 to December 2024. Data were collected on demographics, clinical presentation, imaging, surgical management, staging, histopathological risk categorization, and outcomes. Management followed the UMBRELLA SIOP-RTSG 2016 protocol.
Results:
Twenty-eight patients included in the cohort in which 86% (24) had unilateral while 14% (4) patients had bilateral lesions. The median age was 27 months, with a male-to-female ratio of 2.5:1. Abdominal mass was the most common presentation (93%) and 21% had hypertension. Surgeries included nephroureterectomy, nephron-sparing surgery (22%), and venotomy in tumor thrombus cases. Histology revealed intermediate risk in 78%, high risk in 18%, and low risk in 4%. At 1-year, OS and event-free survival (EFS) were 96% and 87%, respectively. At 3 years, OS and EFS were 85% and 82%. Survival was 100% in children <5 years and for bilateral tumors, but significantly lower in children >5 years. Two patients experienced recurrence. Two died from febrile neutropenia. Follow-up ranged from 4 to 64 months (Median 20.5 months).
Conclusion:
Despite limited resources, adherence to risk-adapted SIOP protocols led to favorable survival outcomes, even in complex presentations such as vascular thrombus or bilateral tumors. Early diagnosis, robust chemotherapy support, and surgical expertise remain the key to improved outcomes in LMIC settings.
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