title: Influence of image-defined risk factors on the outcome of patients with localised neuroblastoma. A report from the LNESG1 study of the European International Society of Paediatric Oncology Neuroblastoma Group.
abstract: BACKGROUND
  The European multicenter study LNESG1 was designed to evaluate the safety and efficacy of surgical treatment alone in patients with localised neuroblastoma. In a retrospective, observational study we examined the impact of image-defined risk factors (IDRF) on operative complications and survival (EFS and OS).
  
  
  METHODS
  534 patients with localised, non-MYCN amplified neuroblastoma were recruited between 1995 and 1999. Group 1 consisted of 291 patients without IDRF (Stage L1 in the International Neuroblastoma Risk Group (INRG) staging system), all treated with primary surgery. Group 2: 118 patients with IDRF (INRG Stage L2), also treated with primary surgery. Group 3: 125 patients in whom primary surgery was not attempted, 106 receiving neo-adjuvant chemotherapy.
  
  
  RESULTS
  In L1 patients (Group 1) 5-year EFS was 92% and OS 98%. In L2 patients (Group 2 and 3) EFS was 79% and OS 89%. The differences in both EFS and OS were significant. EFS and OS in Group 2 (86% and 95%) were significantly better than 73% and 83% in Group 3. In INSS stage 1, 2 and 3, EFS were respectively 94%, 81% and 76%. Except between stage 2 and 3 the differences were significant. OS were respectively 99%, 93% and 83%, all significantly different. The 17% operative complication rate in L2 patients was significantly higher than 5% in L1 patients.
  
  
  CONCLUSIONS
  In localised neuroblastoma, IDRF at diagnosis are associated with worse survival rates and higher rates of operative complications. The impact of IDRF should become an integrated part of therapy planning.
authors: Department of Hepatic, Gastrointestinal and Paediatric surgery, Oslo University Hospital - Rikshospitalet, Oslo, Norway.
authors: Tom
authors: T
authors: Monclair
authors: Service of Statistics, Institut Curie, Paris, France.
authors: Véronique
authors: V
authors: Mosseri
authors: Pediatric Surgery Unit-Women's and Children Health Department, University Hospital of Padua, Italy.
authors: Giovanni
authors: G
authors: Cecchetto
authors: Department of Paediatric Haematology-Oncology, Giannina Gaslini Children's Hospital, Genova, Italy.
authors: Bruno
authors: B
authors: De Bernardi
authors: Department of Paediatric Oncology, Institut Curie, Paris, France.
authors: Jean
authors: J
authors: Michon
authors: Department of Paediatric Surgery, St Georges Hospital, London, United Kingdom.
authors: Keith
authors: K
authors: Holmes
mesh_terms: D000008:Abdominal Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003952:Diagnostic Imaging / Q000379:methods; D005060:Europe / Q000453:epidemiology; D005260:Female; D016259:Genes, myc; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D015337:Multicenter Studies as Topic / Q000706:statistics & numerical data*; D064847:Multimodal Imaging; D020360:Neoadjuvant Therapy; D009361:Neoplasm Invasiveness; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D011183:Postoperative Complications / Q000453:epidemiology / Q000517:prevention & control; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013899:Thoracic Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents
keywords: localised neuroblastoma; risk factors; surgery