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

Pediatr Blood Cancer. 2015 Sep;62(9):1536-42. doi: 10.1002/pbc.25460. Epub 2015 Feb 8.

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).

Procedure: 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.

Keywords: localised neuroblastoma; risk factors; surgery.

Publication types

  • Observational Study

MeSH terms

  • Abdominal Neoplasms / diagnosis
  • Abdominal Neoplasms / drug therapy
  • Abdominal Neoplasms / epidemiology
  • Abdominal Neoplasms / pathology
  • Abdominal Neoplasms / surgery
  • Adolescent
  • Antineoplastic Agents / administration & dosage
  • Antineoplastic Agents / therapeutic use
  • Child
  • Child, Preschool
  • Diagnostic Imaging* / methods
  • Europe / epidemiology
  • Female
  • Genes, myc
  • Head and Neck Neoplasms / diagnosis
  • Head and Neck Neoplasms / drug therapy
  • Head and Neck Neoplasms / epidemiology
  • Head and Neck Neoplasms / pathology
  • Head and Neck Neoplasms / surgery
  • Humans
  • Infant
  • Infant, Newborn
  • Kaplan-Meier Estimate
  • Male
  • Multicenter Studies as Topic / statistics & numerical data*
  • Multimodal Imaging
  • Neoadjuvant Therapy
  • Neoplasm Invasiveness
  • Neoplasm Staging
  • Neuroblastoma / diagnosis
  • Neuroblastoma / drug therapy
  • Neuroblastoma / epidemiology*
  • Neuroblastoma / pathology
  • Neuroblastoma / surgery
  • Postoperative Complications / epidemiology
  • Postoperative Complications / prevention & control
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Thoracic Neoplasms / diagnosis
  • Thoracic Neoplasms / drug therapy
  • Thoracic Neoplasms / epidemiology
  • Thoracic Neoplasms / pathology
  • Thoracic Neoplasms / surgery
  • Treatment Outcome

Substances

  • Antineoplastic Agents