title: Pretreatment for bilateral nephroblastomatosis is an independent risk factor for progressive disease in patients with stage V nephroblastoma.
abstract: BACKGROUND
  Treatment of stage V nephroblastoma is less established and more complex than in unilateral nephroblastoma.
  
  
  METHODS
  Retrospective analysis of 121 consecutive patients with stage V nephroblastoma registered from January 1989 to May 2005. Registration, prospective data collection and treatment were carried out within the framework of 3 consecutive SIOP/GPOH-nephroblastoma-trials.
  
  
  RESULTS
  19 patients had metastasis and 29 syndromes at diagnosis. 13 patients had been pretreated for bilateral nephroblastomatosis. 1 patient was not treated and 17 patients had upfront surgery. Preoperative treatment duration ranged from 1-12 weeks (n=103). 1-3 preoperative treatment-cycles resulted in average tumor-volume-reduction of 45%. 1 patient underwent bilateral nephrectomy. 52% of the patients had 2 functioning kidneys after the end of treatment. 20 patients had died after mean follow-up of 8.6 years. 5y-Progression-Free (PFS) and Overall-Survival (OS) were excellent for patients having a localized disease without pretreatment for nephroblastomatosis (5yPFS/OS: 80±4%/93±3%). Metastasis at diagnosis (51±12%/56±12%; p=0.003) and pretreatment for nephroblastomatosis (37±14%/67±13%; p<0.001) were associated with significantly poorer outcome. Cox-regression analysis revealed an independent influence of pretreatment for nephroblastomatosis, metastasis and syndromes on PFS. The latter 2 as well as anaplasia and age (<2 years or >3 years) had an independent influence on OS.
  
  
  CONCLUSIONS
  Pretreatment for nephroblastomatosis, metastasis and syndromes are independent risk factors. 1-3 preoperative treatment-cycles are sufficient to achieve save nephron-sparing-surgery in most patients.
authors.affiliation: Saarland University Hospital, Pediatric Hematology and Oncology, -Homburg/Saar, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Furtwängler
authors.affiliation: Saarland University Hospital, Pediatric Hematology and Oncology, -Homburg/Saar, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Schmolze
authors.affiliation: Dep. of Biometry, Epidemiology and medical Informatics, Saarland -University, Homburg, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Gräber
authors.affiliation: Paidopathology, Schleswig Holstein University Hospital, Campus Kiel, Kiel, Germany.
authors.forename: I
authors.initials: I
authors.lastname: Leuschner
authors.affiliation: Paidopathology, Vienna University, Vienna, Austria.
authors.forename: G
authors.initials: G
authors.lastname: Amann
authors.affiliation: Diagnostic Radiology, Division of Pediatric Radiology, Heidelberg, Germany.
authors.forename: J-P
authors.initials: JP
authors.lastname: Schenk
authors.affiliation: Children's Hospital, University Hospital, Zurich, Switzerland.
authors.forename: F
authors.initials: F
authors.lastname: Niggli
authors.affiliation: Pediatric Hematology and Oncology, St. Anna Forschungspital, Vienna, Austria.
authors.forename: L
authors.initials: L
authors.lastname: Kager
authors.affiliation: Pediatric Surgery, LMU Hospital, München, Germany.
authors.forename: D
authors.initials: D
authors.lastname: von Schweinitz
authors.affiliation: Saarland University Hospital, Pediatric Hematology and Oncology, -Homburg/Saar, Germany.
authors.forename: N
authors.initials: N
authors.lastname: Graf
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects* / Q000627:therapeutic use; D002471:Cell Transformation, Neoplastic / Q000187:drug effects* / Q000473:pathology; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003609:Dactinomycin / Q000008:administration & dosage / Q000009:adverse effects; D018450:Disease Progression; D004317:Doxorubicin / Q000008:administration & dosage / Q000009:adverse effects; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000187:drug effects / Q000473:pathology; D007680:Kidney Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D020360:Neoadjuvant Therapy / Q000009:adverse effects*; D009367:Neoplasm Staging; D009378:Neoplasms, Multiple Primary / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D016609:Neoplasms, Second Primary / Q000139:chemically induced / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D009392:Nephrectomy; D015996:Survival Rate; D047368:Tumor Burden; D014750:Vincristine / Q000008:administration & dosage / Q000009:adverse effects; D009396:Wilms Tumor / Q000401:mortality / Q000473:pathology / Q000628:therapy*
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin