SF-1 overexpression in childhood adrenocortical tumours

• Published in: European journal of cancer (1990) 2006-05, Vol.42 (8), p.1040-1043
• Main Authors: Pianovski, Mara A.D., Cavalli, Luciane R., Figueiredo, Bonald C., Santos, Savana C.L., Doghman, Mabrouka, Ribeiro, Raul C., Oliveira, Antonio G., Michalkiewicz, Edson, Rodrigues, Giovanna A., Zambetti, Gerard, Haddad, Bassem R., Lalli, Enzo
• Format: Article
• Language: English
• Subjects: Adrenal cortex; Adrenal Cortex Neoplasms; Adrenal Cortex Neoplasms - genetics; Adrenal Cortex Neoplasms - metabolism; Biochemistry, Molecular Biology; Biological and medical sciences; Cancer; Case-Control Studies; Child; Child, Preschool; Childhood tumours; Diploidy; Female; FISH; Germ-Line Mutation; Germ-Line Mutation - genetics; Homeodomain Proteins; Homeodomain Proteins - genetics; Homeodomain Proteins - metabolism; Human health and pathology; Humans; In Situ Hybridization, Fluorescence; Infant; Life Sciences; Loss of Heterozygosity; Male; Medical sciences; Neoplasm Proteins; Neoplasm Proteins - genetics; Neoplasm Proteins - metabolism; Pharmacology. Drug treatments; Receptors, Cytoplasmic and Nuclear; Receptors, Cytoplasmic and Nuclear - genetics; Receptors, Cytoplasmic and Nuclear - metabolism; Steroidogenic Factor 1; Steroidogenic factor 1 (SF-1); Transcription Factors; Transcription Factors - genetics; Transcription Factors - metabolism; Tumors
• ISSN: 0959-8049; 1879-0852
• DOI: 10.1016/j.ejca.2006.01.022

The steroidogenic factor 1 ( SF- 1) gene encodes a transcription factor playing a pivotal role in the regulation of adrenogenital development. We have recently shown that SF- 1 is amplified in childhood adrenocortical tumours (ACT). This study was aimed to assess if an increase in SF- 1 gene copy number was associated with increased protein levels and to study the correlation between SF- 1 expression and ACT clinical parameters. An increased SF- 1 copy number was detected in eight of the 10 ACT cases studied. Conversely, the SF-1 protein was found to be overexpressed in all cases, compared to normal age-matched adrenal glands. No significant correlation was found between SF-1 protein levels and its gene copy number. Furthermore, no significant correlation existed with histological grade or with the clinical manifestation or evolution of disease. This data show that SF- 1 overexpression is widespread in childhood ACT and is likely to play a role in its pathogenesis.