Mortality rate of adrenocortical tumors in children under 15 years of age in Curitiba, Brazil

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.

Open link

Publication date

January 2006

DOI

10.1002/pbc.20624

ISSN

1545-5009

Citation count (estimate)

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 ...