title: Management and outcome of stage 3 neuroblastoma.
abstract: OBJECTIVE
  The management of patients with International Neuroblastoma Staging System (INSS) stage 3 neuroblastoma (NB) is not consistent worldwide. We describe a single centre approach at Memorial Sloan-Kettering Cancer Centre (MSKCC) from 1991 to 2007 that minimises therapy except for those patients with MYCN-amplified NB.
  
  
  METHODS
  In this retrospective analysis of 69 patients, tumour MYCN was not amplified in 53 and amplified in 16. Event-free survival (EFS) and overall survival (OS) were determined by Kaplan-Meier analysis.
  
  
  RESULTS
  Fourteen patients with non-MYCN-amplified tumours were treated with surgery alone (group A) and the remaining 39 (group B) with surgery following chemotherapy that was initiated and administered at non-MSKCC institutions. Chemotherapy was discontinued after surgery in 38/39 of the latter. The 10-year EFS and OS for all patients with MYCN-non-amplified NB were 74.9+/-16.9% and 92.6+/-5.5%, respectively. There was no difference in OS between groups A and B (p=0.2; 10-year OS for groups A and B was 84.6+/-14% and 97.1+/-2.9%, respectively). Patients with MYCN-amplified disease (group C) underwent dose-intensive induction, tumour resection and local radiotherapy: 13 achieved complete or very good partial remission, and 10 received myeloablative chemotherapy. 11/16 patients also received 3F8-based immunotherapy: 10 remain free of disease. The 10-year EFS and OS for patients with MYCN-amplified neuroblastoma treated with immunotherapy were both 90.9+/-8.7%.
  
  
  CONCLUSIONS
  Patients with MYCN-non-amplified stage 3 NB can be successfully treated with surgery without the need for radiotherapy or continuation of chemotherapy. Combination of dose-intensive chemotherapy, surgery, radiotherapy and immunotherapy was associated with a favourable outcome for most patients with MYCN-amplified stage 3 NB.
authors.affiliation: Department of Paediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA. modaks@mskcc.org
authors.forename: Shakeel
authors.initials: S
authors.lastname: Modak
authors.forename: Brian H
authors.initials: BH
authors.lastname: Kushner
authors.forename: Michael P
authors.initials: MP
authors.lastname: LaQuaglia
authors.forename: Kim
authors.initials: K
authors.lastname: Kramer
authors.forename: Nai-Kong V
authors.initials: NK
authors.lastname: Cheung
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005784:Gene Amplification; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D000071447:N-Myc Proto-Oncogene Protein; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000235:genetics / Q000401:mortality / Q000628:therapy*; D009687:Nuclear Proteins / Q000235:genetics; D015513:Oncogene Proteins / Q000235:genetics; D012189:Retrospective Studies; D015996:Survival Rate
chemical_list: D000970:Antineoplastic Agents; C459801:MYCN protein, human; D000071447:N-Myc Proto-Oncogene Protein; D009687:Nuclear Proteins; D015513:Oncogene Proteins