Protocol-based Management of Nephroblastoma: Five-year Outcomes with SIOP-RTSG Guidelines in an Indian Cohort

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.