title: Clinical prognostic factors in 1277 patients with neuroblastoma: results of The European Neuroblastoma Study Group 'Survey' 1982-1992.
abstract: In 1982 the European Neuroblastoma Study Group (ENSG) established a prospective registry for patients with newly diagnosed neuroblastoma ('The ENSG Survey'). Clinical information was collected primarily to: (a) establish an ENSG database; and (b) investigate prognostic factors in neuroblastoma. This paper summarises the results of the survey. By 1992, 1277 patients with a median age of 26 months (range: 0-289 months), gender ratio of 1.19 M:F had been registered from 30 centres. The median follow-up of survivors is 9.7 years (range: 1-14 years). Overall 5-year survival (S) is 45% (95% CI 42-48%), and event-free survival (EFS) is 43% (95% CI 40-45%). For both survival and EFS the key established prognostic factors, stage and age, are highly significant (P<0.001). In particular, patients under 1 year of age at diagnosis, whatever the disease stage, had a more favourable prognosis than older patients; stage 2 (EFS 93% (95% (CI 85-97) versus 76% (95% CI 67-86), P=0.02), stage 3 (EFS 91% (95% CI 82-96) versus 52% (95% CI 44-60), P<0.001) and stage 4 (EFS 59% (95% CI 48-69) versus 16% (95% CI 13-19), P<0.001). Multivariate analysis established that the anatomical location of the primary tumour (i.e. abdominal versus other sites) and primary tumour volume also conferred a statistically significant difference. In stage 4 disease the 20% of patients without demonstrable bone marrow involvement had a more favourable prognosis than those with infiltrated marrow (EFS 36% (95% CI 13-19) versus 16% (95% CI 29-45), P<0.001). Urine catecholamine metabolite levels (raised versus normal), histology (ganglioneuroblastoma versus neuroblastoma) and gender had no significant effect on outcome after stage and age were accounted for. 5-year survival following first relapse is only 5.6% (95% CI 2.8-8.4). This ENSG Survey provides secure data for future comparisons with new prognostic factors and treatment programmes.
authors.affiliation: Sir James Spence Institute of Child Health, University of Newcastle upon Tyne, Newcastle upon Tyne, UK. s.j.cotterill@ncl.ac.uk
authors.forename: S J
authors.initials: SJ
authors.lastname: Cotterill
authors.forename: A D
authors.initials: AD
authors.lastname: Pearson
authors.forename: J
authors.initials: J
authors.lastname: Pritchard
authors.forename: A B
authors.initials: AB
authors.lastname: Foot
authors.forename: B
authors.initials: B
authors.lastname: Roald
authors.forename: J A
authors.initials: JA
authors.lastname: Kohler
authors.forename: J
authors.initials: J
authors.lastname: Imeson
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000367:Age Factors; D000368:Aged; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005060:Europe / Q000453:epidemiology; D005260:Female; D018305:Ganglioneuroblastoma / Q000401:mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local / Q000401:mortality; D009367:Neoplasm Staging / Q000379:methods; D009447:Neuroblastoma / Q000401:mortality*; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D017678:Sex Distribution