Natural history and biology of stage A neuroblastoma: a Pediatric Oncology Group Study

J Pediatr Hematol Oncol. May-Jun 2000;22(3):197-205. doi: 10.1097/00043426-200005000-00003.

Abstract

Purpose: To prospectively analyze the outcome of patients with Stage A neuroblastoma (NB) treated with surgery alone, especially with regard to the prognostic significance of age, tumor site, MYCN copy number, tumor cell ploidy, and histology.

Patients and methods: The clinical course of 329 patients with Stage A disease registered on the POG NB Biology Study #9047 between February, 1990 and October, 1997 were evaluated. Age, tumor site, MYCN copy number, tumor cell ploidy, and histology were analyzed for their impact on event-free survival (EFS) and survival (S).

Results: The 5-year estimated EFS and S rates for the 329 patients were 91% (+/-3%) and 96% (+/-2%), respectively. The EFS rate was similar for infants younger than 12 months and children age 12 months or older, but age older than 12 months was predictive of lower S rates (P = 0.044). Patients with adrenal, abdominal non-adrenal, thoracic, and cervical tumors had similar S rates. The majority of patients had tumors with favorable biologic features, and only 3% had MYCN amplification. For infants with diploid tumors, the EFS rate was 82% (+/-16%), but effective therapy yielded an S rate of 100%. Rate of S was 80% (+/-26%) and 64% (+/-27%) for patients with unfavorable tumor histology and MYCN-amplified tumors, respectively.

Conclusion: The outcome for patients with Stage A NB treated with surgery alone is excellent. Although EFS and S rates were significantly worse for patients with MYCN-amplified tumors, a subset achieved long-term remission after surgery alone. For patients with Stage A and MYCN amplification, additional factors are needed to distinguish the patients who will achieve long-term remission with surgery alone from those who will develop recurrent disease.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Abdominal Neoplasms / diagnostic imaging
  • Abdominal Neoplasms / epidemiology
  • Abdominal Neoplasms / genetics
  • Abdominal Neoplasms / pathology
  • Abdominal Neoplasms / surgery
  • Adolescent
  • Adrenal Gland Neoplasms / epidemiology
  • Adrenal Gland Neoplasms / genetics
  • Adrenal Gland Neoplasms / pathology
  • Adrenal Gland Neoplasms / surgery
  • Adrenal Gland Neoplasms / ultrastructure
  • Adult
  • Age Factors
  • Child
  • Child, Preschool
  • Cohort Studies
  • DNA, Neoplasm / genetics
  • Disease-Free Survival
  • Female
  • Fetal Diseases / diagnostic imaging
  • Fetal Diseases / epidemiology
  • Gene Amplification
  • Genes, myc
  • Head and Neck Neoplasms / epidemiology
  • Head and Neck Neoplasms / genetics
  • Head and Neck Neoplasms / pathology
  • Head and Neck Neoplasms / surgery
  • Head and Neck Neoplasms / ultrastructure
  • Humans
  • Infant
  • Infant, Newborn
  • Life Tables
  • Male
  • Neoplasm Metastasis
  • Neoplasm Recurrence, Local / epidemiology
  • Neoplasm Staging
  • Neuroblastoma / diagnostic imaging
  • Neuroblastoma / epidemiology*
  • Neuroblastoma / genetics
  • Neuroblastoma / pathology
  • Neuroblastoma / surgery
  • Organ Specificity
  • Ploidies
  • Prognosis
  • Prospective Studies
  • Remission Induction
  • Survival Analysis
  • Thoracic Neoplasms / epidemiology
  • Thoracic Neoplasms / genetics
  • Thoracic Neoplasms / pathology
  • Thoracic Neoplasms / surgery
  • Thoracic Neoplasms / ultrastructure
  • Treatment Outcome
  • Ultrasonography, Prenatal

Substances

  • DNA, Neoplasm