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Horm Metab Res 2007; 39(6): 461-466
DOI: 10.1055/s-2007-981476
Review

© Georg Thieme Verlag KG Stuttgart · New York

The Molecular Pathogenesis of Childhood Adrenocortical Tumors

M. Q. Almeida 1 , A. C. Latronico 1
  • 1Unidade de Endocrinologia do Desenvolvimento e Laboratório de Hormônios e Genética Molecular LIM-42, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brasil
Further Information

Publication History

received 1.11.2006

accepted 18.4.2007

Publication Date:
18 June 2007 (online)

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Abstract

Adrenocortical tumors in children and adolescents are rare events. However, the high incidence of adrenocortical tumors in children from the Southern region of Brazil is particularly remarkable, since it has been estimated to be approximately 10-15 times greater than the worldwide incidence. To date, there are no histological or molecular markers that can reliably distinguish benign from malignant adrenocortical tumors. The study of rare genetic syndromes associated with adrenocortical tumors has greatly contributed to the elucidation of sporadic adrenocortical tumorigenesis. Recently, considerable advances toward understanding the molecular mechanisms of adrenocortical tumorigenesis in Brazilian children and adolescents with sporadic adrenocortical tumors have been made. Some of the molecular aspects of sporadic adrenocortical tumors arising in children and adolescents are reviewed here.

Key words

Adrenocortical tumorigenesis - children - p53 - IGF2 - SF-1

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Correspondence

A. C. LatronicoMD 

Unidade de Endocrinologia do Desenvolvimento e Laboratório de Hormônios e Genética Molecular LIM-42

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Email: madsonalmeida@gmail.com