title: Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups.
abstract: BACKGROUND
  To review the clinical characteristics and survival of infants diagnosed with a primary renal tumor in the first 7 months of life.
  
  
  METHODS
  A retrospective data review of patients registered in five large international protocols (SFOP/GPOH/SIOP9/93-01, UKW3 and NWTSG 4 and 5) spanning 1985-2002.
  
  
  RESULTS
  750 (7.2%) of 10,430 registered patients were diagnosed with a renal tumor before age 213 days. Tumor types were Wilms tumor (WT) 58%; congenital mesoblastic nephroma (CMN) 18%; malignant rhabdoid tumor (MRTK) 8%; clear cell sarcoma (CCSK) 2%; non-Wilms tumor (unspecified) 6%; histology unknown, 9%. CMN predominated among tumors diagnosed in the first month of life (54%) but its relative contribution diminished to <10% of all cases diagnosed after the age of 3 months (P < 0.001). Among 639 cases with specified histology and stage, 9/11 stage IV tumors were MRTK, 37/39 bilateral tumors were WT. In 626 children where surgical approach was specified, 522 had immediate nephrectomy. For all cases, 5 years event-free survival (EFS) was 80% and overall survival (OS) 86%. Five years EFS and OS respectively by tumor type were WT (86%, 93%), CMN (94%, 96%), CCSK (49%, 51%), MRTK (16%, 16%).
  
  
  CONCLUSIONS
  Renal tumors diagnosed in the first 7 months of life generally have an excellent prognosis though histology is an important prognostic factor. In the first 2 months of life the prevalence of CMN is high. The relative occurrence of WT increases rapidly with age thereafter. Bilateral tumors are usually WT. Tumors with metastases at diagnosis are usually MRTK.
authors: Department of Pediatric Oncology/Hematology; Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands. m.vandenheuvel@erasmusmc.nl
authors: Marry M
authors: MM
authors: van den Heuvel-Eibrink
authors: Paul
authors: P
authors: Grundy
authors: Norbert
authors: N
authors: Graf
authors: Kathy
authors: K
authors: Pritchard-Jones
authors: Christophe
authors: C
authors: Bergeron
authors: Catherine
authors: C
authors: Patte
authors: Harm
authors: H
authors: van Tinteren
authors: Annie
authors: A
authors: Rey
authors: Carolyn
authors: C
authors: Langford
authors: Jim R
authors: JR
authors: Anderson
authors: Jan
authors: J
authors: de Kraker
mesh_terms: D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007680:Kidney Neoplasms / Q000151:congenital / Q000175:diagnosis* / Q000401:mortality; D008297:Male; D015996:Survival Rate; D009396:Wilms Tumor / Q000151:congenital / Q000175:diagnosis* / Q000401:mortality