title: Mortality rate of adrenocortical tumors in children under 15 years of age in Curitiba, Brazil.
abstract: BACKGROUND
  Several reports refer to an increased frequency of adrenal cortex tumors (ACT) among children in Southern Brazil, yet all data have been derived from hospital-based registries. An inherited germline mutation in the p53 gene (TP53 R337H) is detected in virtually all children with ACT in this region and accounts for the excess cases observed.
  
  
  METHODS
  We reviewed all death certificates that mentioned ACT or adrenal neuroblastoma (NB) and which were reported to the Paraná State Department of Health between 1998 and 2003, for individuals younger than 15 years who resided in the Curitiba metropolitan region.
  
  
  RESULTS
  Eight deaths from ACT and ten from NB were identified. The age-standardized mortality rate per million children <15 years of age in the Curitiba metropolitan region during the years 1998-2003 was 1.6 (95% confidence interval (CI) 1.4, 1.8) for ACT and 2.3 (95% CI 2.0, 2.5) for NB. The ratio of the adrenal NB and ACT age-adjusted mortality rates was 1.43. The incidence of ACT estimated from the mortality data, assuming an ACT survival rate of 0.542, was 3.5 (95% CI 2.9, 4.2).
  
  
  CONCLUSIONS
  Our investigation of population-based mortality confirms the evidence from hospital-based registries of a clustering of ACT in Southern Brazil. In addition, our data suggest that the incidence of ACT in this region is about 12-18 times the incidence reported in the United States and Europe.
authors.affiliation: Department of Pediatric, Hematology and Pediatric Oncology Division, Clínicas Hospital of the Federal University of Paraná, Brazil.
authors.forename: Mara A D
authors.initials: MA
authors.lastname: Pianovski
authors.forename: Eliane M C P
authors.initials: EM
authors.lastname: Maluf
authors.forename: Denise S
authors.initials: DS
authors.lastname: de Carvalho
authors.forename: Raul C
authors.initials: RC
authors.lastname: Ribeiro
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
authors.forename: Paolo
authors.initials: P
authors.lastname: Boffetta
authors.forename: Patrícia
authors.initials: P
authors.lastname: Zancanella
authors.forename: Bonald C
authors.initials: BC
authors.lastname: Figueiredo
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000453:epidemiology / Q000235:genetics / Q000401:mortality*; D001938:Brazil / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D016158:Genes, p53 / Q000235:genetics; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D009154:Mutation; D009447:Neuroblastoma / Q000401:mortality; D015996:Survival Rate