title: Management and outcomes in massive bilateral Wilms' tumors.
abstract: OBJECTIVE
  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.
  
  
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Sandeep
authors.initials: S
authors.lastname: Agarwala
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Deepak
authors.initials: D
authors.lastname: Mittal
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Veereshwar
authors.initials: V
authors.lastname: Bhatnagar
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: M
authors.initials: M
authors.lastname: Srinivas
authors.affiliation: Department of Medical Oncology, BRAIRCH, AIIMS, New Delhi, India.
authors.forename: Sameer
authors.initials: S
authors.lastname: Bakhshi
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Minu
authors.initials: M
authors.lastname: Bajpai
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Devendra Kumar
authors.initials: DK
authors.lastname: Gupta
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: V K
authors.initials: VK
authors.lastname: Iyer
authors.affiliation: Department of Radiotherapy, BRAIRCH, AIIMS, New Delhi, India.
authors.forename: Bidyut K
authors.initials: BK
authors.lastname: Mohanti
authors.affiliation: Department of Radiodiagnosis, BRAIRCH, AIIMS, New Delhi, India.
authors.forename: Sanjay
authors.initials: S
authors.lastname: Thulkar
keywords: Bilateral Wilms’ tumor; chemotherapy; nephron-sparing surgery; partial nephrectomy; tumorectomy