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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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 ORIGINAL ARTICLE
Year : 2014  |  Volume : 19  |  Issue : 4  |  Page : 208-212

Management and outcomes in massive bilateral Wilms' tumors


1 Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Medical Oncology, BRAIRCH, AIIMS, New Delhi, India
3 Department of Radiotherapy, BRAIRCH, AIIMS, New Delhi, India
4 Department of Radiodiagnosis, BRAIRCH, AIIMS, New Delhi, India

Correspondence Address:
Sandeep Agarwala
Professor, Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi-110029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.142005

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Purpose: To evaluate the outcome of children with bilateral Wilms' tumor (BWT) treated on All India Institute of Medical Sciences-Wilms Tumor-99 (AIIMS-WT-99) protocol. Materials and Methods : All children with BWT, registered in our solid tumor clinic from August 1999 through December 2010 were included. Results: Of the 178 fresh cases of Wilms Tumor (WT) treated during this period, 11 (6.2%) had bilateral involvement. All patients except one (12 and 3 cm), had massive bilateral tumors of more than 10 cm on each side. There were eight boys and three girls in the age range 6-30 months. One patient had Denys-Drash syndrome. Twenty renal units were operated upon (12 tumorectomy, five partial nephrectomy, and three nephrectomies), while one patient with inferior vena cava (IVC) thrombus died of renal failure. Tumor spill occurred in three units, lymphnode was positive in two patients. Local recurrence occurred in four patients (six of 18 renal units (33%)-two bilateral and two unilateral). There was one recurrence in the liver that was treated with radio-frequency ablation. The 5-year overall survival (OS) was 90% (95% confidence interval (CI) = 50.8-98.6) and the relapse free survival (RFS) was 38% (95% CI = 6.1-71.6). Conclusion: Massive BWT respond poorly to preoperative chemotherapy, are often not amenable to partial nephrectomy/tumorectomy and have a higher local recurrence rate, giving a poor RFS.






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