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Mortality rate of adrenocortical tumors in children under 15 years of age in Curitiba, Brazil
Author(s) -
Pianovski Mara A.D.,
Maluf Eliane M.C.P.,
de Carvalho Denise S.,
Ribeiro Raul C.,
RodriguezGalindo Carlos,
Boffetta Paolo,
Zancanella Patrícia,
Figueiredo Bonald C.
Publication year - 2006
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20624
Subject(s) - medicine , curitiba , demography , incidence (geometry) , confidence interval , population , pediatrics , mortality rate , rate ratio , adrenocortical carcinoma , environmental health , humanities , philosophy , physics , sociology , optics
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. Procedure 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. © 2005 Wiley‐Liss, Inc.