title: Neuroblastoma preoperatively treated as nephroblastoma: does inadequate therapy worsen the prognosis?
abstract: BACKGROUND
  The current standard in the treatment of nephroblastoma is preoperative chemotherapy based on radiological appearance. After subsequent surgical removal few tumours proved histologically to be neuroblastoma. We asked whether initial chemotherapy according to nephroblastoma trials would change the prognosis for those neuroblastoma patients.
  
  
  RESULTS
  Out of 1603 patients registered in the German neuroblastoma trials, 29 patients (1.8 %) have preoperatively been treated according nephroblastoma protocols. Advanced stages (11 stage 3, 12 stage 4) were dominant. Diagnostic work up of those patients revealed elevation of catecholamine metabolites in only 39 % (compared to 80 % of the control patients) and mIBG uptake in only 71 % (compared to 89 % of the control patients). Elevation of NSE was observed in 92 % of patients (control group 72 %). Patients with preoperative nephroblastoma treatment were older than the patients of the control group. Risk factors like MYCN amplification or elevation of LDH were more often detected. The outcome of the patients with preoperative chemotherapy according nephroblastoma trials was worse than that of the control group, but risk group adapted survival analysis revealed no disadvantage.
  
  
  CONCLUSIONS
  The prognosis of children with neuroblastoma tumours, which have been radiologically classified as nephroblastoma, is inferior compared to the prognosis of patients without preoperative nephroblastoma therapy. The difference appears to be associated rather with more unfavourable biology than with the element "preoperative chemotherapy".
authors.affiliation: Kinderklinik der Universität Köln, Germany. barbara.hero@medizin-uni-koeln.de
authors.forename: B
authors.initials: B
authors.lastname: Hero
authors.forename: N
authors.initials: N
authors.lastname: Graf
authors.forename: T
authors.initials: T
authors.lastname: Simon
authors.forename: A
authors.initials: A
authors.lastname: Weirich
authors.forename: J
authors.initials: J
authors.lastname: Tröger
authors.forename: F
authors.initials: F
authors.lastname: Berthold
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007677:Kidney Function Tests; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D011379:Prognosis; D009396:Wilms Tumor / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000601:surgery