title: Natural history and biology of stage A neuroblastoma: a Pediatric Oncology Group Study.
abstract: OBJECTIVE
  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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Emory University, Atlanta, Georgia, USA.
authors.forename: C S
authors.initials: CS
authors.lastname: Alvarado
authors.forename: W B
authors.initials: WB
authors.lastname: London
authors.forename: A T
authors.initials: AT
authors.lastname: Look
authors.forename: G M
authors.initials: GM
authors.lastname: Brodeur
authors.forename: D H
authors.initials: DH
authors.lastname: Altmiller
authors.forename: P S
authors.initials: PS
authors.lastname: Thorner
authors.forename: V V
authors.initials: VV
authors.lastname: Joshi
authors.forename: S T
authors.initials: ST
authors.lastname: Rowe
authors.forename: M B
authors.initials: MB
authors.lastname: Nash
authors.forename: E I
authors.initials: EI
authors.lastname: Smith
authors.forename: R P
authors.initials: RP
authors.lastname: Castleberry
authors.forename: S L
authors.initials: SL
authors.lastname: Cohn
mesh_terms: D000008:Abdominal Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000601:surgery / Q000648:ultrastructure; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004273:DNA, Neoplasm / Q000235:genetics; D018572:Disease-Free Survival; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology; D005784:Gene Amplification; D016259:Genes, myc; D006258:Head and Neck Neoplasms / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000601:surgery / Q000648:ultrastructure; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015993:Life Tables; D008297:Male; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D009928:Organ Specificity; D011003:Ploidies; D011379:Prognosis; D011446:Prospective Studies; D012074:Remission Induction; D016019:Survival Analysis; D013899:Thoracic Neoplasms / Q000453:epidemiology / Q000235:genetics / Q000473:pathology / Q000601:surgery / Q000648:ultrastructure; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal
chemical_list: D004273:DNA, Neoplasm