title: Heterogeneity of disease classified as stage III in Wilms tumor: a report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP).
abstract: OBJECTIVE
  We analyzed whether the prognosis can differ among Wilms tumors (WT) labeled as Stage III according to currently adopted classification systems.
  
  
  METHODS
  Patients with nonanaplastic Stage III WT consecutively registered in two Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials (CNR-92, TW-2003) were the subjects in the present analysis. The steady mainstay of therapy was primary nephrectomy, followed by three-drug chemotherapy with vincristine, dactinomycin, doxorubicin, and abdominal radiotherapy (RT).
  
  
  RESULTS
  Ninety-nine WT patients met the criteria for classification as Stage III according to a revised version of the National Wilms Tumor Study-3 staging system (51 patients in CNR-92, 48 patients in TW-2003). Regional lymph nodes (LN) were not biopsied in 16 patients. After a median follow-up of 66 months, the 4-year disease-free survival (DFS) and overall survival (OS) rates were 85% ± 4% and 92% ± 3%, respectively, for the whole group. For 38 children with positive LN, the 4-year DFS rate was 73% ± 7%, as opposed to 98% ± 2% for the 45 children with Stage III WT according to the other criteria but with negative biopsied LN (p = 0.001). The subgroup with the worst prognosis consisted of children more than 2 years old with positive LN (DFS 67% ± 8%). A delay between surgery and RT > 30 days had an adverse impact on the abdominal tumor relapse rate.
  
  
  CONCLUSIONS
  This study provides further evidence that Stage III tumors with LN metastases might be distinguished from WTs meeting the other criteria for classification as Stage III. The worse outcome of the former may warrant a prospective study on the effects of intensified therapy. A subclassification of Stage III tumors is discussed.
authors.affiliation: Pediatric Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy. filippo.spreafico@istitutotumori.mi.it
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.forename: Monica
authors.initials: M
authors.lastname: Terenziani
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Maurizio
authors.initials: M
authors.lastname: Bianchi
authors.forename: Massimo
authors.initials: M
authors.lastname: Provenzi
authors.forename: Paolo
authors.initials: P
authors.lastname: Indolfi
authors.forename: Andrea
authors.initials: A
authors.lastname: Pession
authors.forename: Marilina
authors.initials: M
authors.lastname: Nantron
authors.forename: Andrea
authors.initials: A
authors.lastname: Di Cataldo
authors.forename: Alfonso
authors.initials: A
authors.lastname: Marchianò
authors.forename: Serena
authors.initials: S
authors.lastname: Catania
authors.forename: Franca
authors.initials: F
authors.lastname: Fossati Bellani
authors.forename: Luigi
authors.initials: L
authors.lastname: Piva
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy / Q000379:methods; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007558:Italy; D007680:Kidney Neoplasms / Q000145:classification / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D008207:Lymphatic Metastasis; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000532:radiotherapy; D009367:Neoplasm Staging / Q000379:methods*; D009392:Nephrectomy; D011379:Prognosis; D011879:Radiotherapy Dosage; D012955:Societies, Medical; D014750:Vincristine / Q000008:administration & dosage; D009396:Wilms Tumor / Q000145:classification / Q000401:mortality / Q000473:pathology* / Q000556:secondary / Q000628:therapy
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin