The molecular pathogenesis of childhood adrenocortical tumors.
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.
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Unidade 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.
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D000306:Adrenal Cortex Neoplasms / Q000209:etiology* / Q000235:genetics; D002874:Chromosome Mapping; D002886:Chromosomes, Human, Pair 17; D019205:GTP-Binding Protein alpha Subunits, Gs / Q000235:genetics; D016158:Genes, p53; D018398:Homeodomain Proteins / Q000235:genetics; D006801:Humans; D007335:Insulin-Like Growth Factor II / Q000235:genetics; D009154:Mutation; D009693:Nucleic Acid Hybridization; D011493:Protein Kinase C / Q000502:physiology; D018043:Receptors, Corticotropin / Q000235:genetics; D018160:Receptors, Cytoplasmic and Nuclear / Q000235:genetics; D054339:Steroidogenic Factor 1; D014157:Transcription Factors / Q000235:genetics
D018398:Homeodomain Proteins; D018043:Receptors, Corticotropin; D018160:Receptors, Cytoplasmic and Nuclear; D054339:Steroidogenic Factor 1; D014157:Transcription Factors; D007335:Insulin-Like Growth Factor II; D011493:Protein Kinase C; D019205:GTP-Binding Protein alpha Subunits, Gs