SF-1 overexpression in childhood adrenocortical tumours.

Creators: 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

Others:: Division of Pediatric Hematology and Oncology, Department of Pediatrics ; Universidade Federal do Paraná (UFPR); Department of Pediatrics (CEGEMPAC) ; Centro de Genética Molecular e Pesquisa do Câncer em Crianças; Department of Oncology ; Lombardi Comprehensive Cancer Center-Georgetown University Medical Center; Institut de pharmacologie moléculaire et cellulaire (IPMC) ; Université Nice Sophia Antipolis (1965 - 2019) (UNS) ; COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Centre National de la Recherche Scientifique (CNRS); Department of Hematology and Oncology and International Outreach Program ; St Jude Children's Research Hospital; Division of Pediatric Surgery ; Centro Infantil Boldrini; Division of Pediatric Oncology Surgery ; Erasto Gaertner Hospital; Department of Biochemistry ; St Jude Children's Research Hospital; CNRS, FRM, ARC, CAPES-COFECUB, Fundação Araucária, Paraná, NIH, American Lebanese Syrian Associated Charities

Description

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.

SF-1 overexpression in childhood adrenocortical tumours.

Description

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