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. 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.
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In Southern Brazil, childhood ACC incidence is 2.9 to 4.2 per million per year, compared with 0.2 to 0.3 per million children per year worldwide.
“In Southern Brazil, the incidence during childhood is 2.9 to 4.2 per million per year compared with an estimated incidence of 0.2 to 0.3 per million children per year worldwide (5).”
From Adrenocortical carcinoma (2014) · cites this paper as [5]
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Self-citation
- Authors
- Pianovski, M. A. D., Maluf, E. M. C. P., de Carvalho, D. S., Ribeiro, R. C., Rodriguez‐Galindo, C., Boffetta, P., Zancanella, P., & Figueiredo, B. C.
- Affiliations
- Hospital de Clínicas Universidade Federal do Paraná (BR)
- St. Jude Children's Research Hospital (US)
- Centre international de recherche sur le cancer (FR)
- Corresponding author
- Figueiredo, Bonald C.
- Venue
- Pediatric Blood & Cancer
- Publisher
- Wiley
- Published
- 2005-09-30
- Volume
- 47
- Issue
- 1
- Pages
- 56-60
- ISSN
- 1545-5009 (ISSN-L)
- 1545-5017
- OpenAlex
- W2092466451
- Cited by
- 57
- References
- 41
- Citations by year
- 2025: 3 · 2024: 3 · 2023: 1 · 2022: 3 · 2020: 3 · 2019: 2 · 2018: 1 · 2017: 2 · 2016: 3 · 2015: 1 · 2014: 3 · 2013: 4 · 2012: 4
- FWCI
- 3.18
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Pediatric Blood & Cancer (publishedVersion)
- PubMed (publishedVersion)
- Archivio istituzionale della ricerca (Alma Mater Studiorum Università di Bologna) (submittedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Neurology › Neuroblastoma Research and Treatments
- Health Sciences › Medicine › Oncology › Global Cancer Incidence and Screening
- Keywords
- adrenocortical tumors, adrenal neuroblastoma, child mortality, p53, incidence estimation, southern Brazil, Curitiba, geographic clustering
- MeSH terms
- Adolescent, Adrenal Cortex Neoplasms, Brazil, Child, Child, Preschool, Humans, Infant, Infant, Newborn, Mutation, Neuroblastoma, Incidence, Survival Rate, Genes, p53
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