title: Synchronous bilateral Wilms tumor: a report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP).
abstract: BACKGROUND
  The optimal management of bilateral Wilms tumor (BWT) is challenging, and their survival is lower than for unilateral tumors. This report discusses a large series of BWTs treated in Italy in the last 2 decades.
  
  
  METHODS
  This analysis concerns patients with synchronous BWT registered at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers between 1990 and 2011; details on their treatment and outcome are presented and discussed.
  
  
  RESULTS
  Ninety BWTs were registered in the AIEOP Wilms tumor database. Preoperative chemotherapy was given for a median 12 weeks before definitive tumor resection was attempted. Forty-eight percent of the patients had preservation of bilateral renal parenchyma. The proportion of bilateral nephron-sparing surgeries was not higher in the 37 patients initially given doxorubicin/vincristine/actinomycin D (32%) than in the 43 children receiving vincristine/actinomycin D alone (58%). The 4-year disease-free survival rate was 66.5% ± 5% and overall survival was 80% ± 5% for the cohort as a whole. The 4-year disease-free survival (overall survival) for 18 children with diffuse anaplasia or postchemotherapy blastemal-type tumors was 51% ± 13% (62% ± 13%), as opposed to 72% ± 3% (88% ± 4%) for 68 children with a favorable histology (log-rank P = .04 [P = .007]).
  
  
  CONCLUSIONS
  These results provide further evidence that the optimal duration and choice of drugs for preoperative chemotherapy remain an open question. Outcome remained significantly worse for BWT than for unilateral Wilms tumor. To enable the conservative treatment of as many affected kidneys as possible, only centers with experience in BWT should manage such cases.
authors: Pediatric Oncology Unit, Department of Pediatrics, II University, Naples, Italy.
authors: Paolo
authors: P
authors: Indolfi
authors: Alessandro
authors: A
authors: Jenkner
authors: Monica
authors: M
authors: Terenziani
authors: Alessandro
authors: A
authors: Crocoli
authors: Annalisa
authors: A
authors: Serra
authors: Paola
authors: P
authors: Collini
authors: Davide
authors: D
authors: Biasoni
authors: Lorenza
authors: L
authors: Gandola
authors: Gianni
authors: G
authors: Bisogno
authors: Giovanni
authors: G
authors: Cecchetto
authors: Martina
authors: M
authors: Di Martino
authors: Paolo
authors: P
authors: D'Angelo
authors: Maurizio
authors: M
authors: Bianchi
authors: Massimo
authors: M
authors: Conte
authors: Alessandro
authors: A
authors: Inserra
authors: Andrea
authors: A
authors: Pession
authors: Filippo
authors: F
authors: Spreafico
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D008297:Male; D011446:Prospective Studies; D014750:Vincristine / Q000008:administration & dosage; D009396:Wilms Tumor / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin