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Amplification of the Steroidogenic Factor 1 Gene in Childhood Adrenocortical Tumors

Department of Pediatrics, Center for Molecular Genetics and Cancer Research in Children (CEGEMPAC) (B.C.F., M.A.D.P., G.A.R.), Department of Pediatrics, Division of Pediatric Hematology and Oncology (M.A.D.P.), and Department of Pediatrics, Division of Pediatric Endocrinology (R.S., L.D.), Federal University of Paraná, Curitiba PR 80.030-110, Brazil; Department of Oncology and Institute for Molecular and Human Genetics/Lombardi Comprehensive Cancer Center (L.R.C., B.R.H.), Georgetown University, Washington, DC 20007; Institut de Génétique et de Biologie Moléculaire et Cellulaire Centre National de Ciencia y Tecnologia–Institut National de la Santé et de la Recherche Médicale (E.L.), Université Louis Pasteur, 67404 Illkirch, France; Departments of Hematology-Oncology (R.C.R.) and Biochemistry (G.Z.) and the International Outreach Program (R.C.R.), St. Jude Children’s Research Hospital, Memphis, Tennessee 38105; and Department of Pediatrics (R.C.R.), University of Tennessee, College of Medicine, Memphis, Tennessee 38163

Address all correspondence and requests for reprints to: Bassem R. Haddad, M.D., Institute for Molecular and Human Genetics, Georgetown University Medical Center, 3800 Reservoir Road NW, Main 4000, Washington, DC 20007. E-mail: haddadb1{at}georgetown.edu.

Southern Brazil has one of the highest incidences of childhood adrenocortical tumors (ACTs), occurring 10–15 times more frequently than worldwide estimates. The reasons for this increase remain elusive. In an attempt to further characterize the genetic changes in childhood ACTs, we recently detected a consistent gain of 9q (or a portion of it) in eight of nine cases of pediatric ACTs and amplification of 9q34 in the majority of these cases using comparative genomic hybridization. Other studies involving both childhood and adult ACTs have corroborated these findings. To follow up on these results, we examined whether the steroidogenic factor 1 (SF-1) gene, which is located in this chromosomal region and plays an important role in the development and function of the adrenal cortex is amplified in these ACT cases. We detected increased copy number of the SF-1 gene in all eight cases with 9q gain, suggesting an association between an increased copy number of the SF-1 gene and adrenocortical tumorigenesis.

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