Abstract | BACKGROUND:
Steroidogenic factor 1 (SF-1) is a key determinant of endocrine development and function of adrenal cortex. SF-1 overexpression and gene amplification were previously demonstrated in a small group of pediatric adrenocortical tumors. OBJECTIVE: Our objective was to determine the frequency of SF-1 protein expression and gene amplification in a large cohort of pediatric and adult adrenocortical tumors. PATIENTS: SF-1 protein expression was assessed in a cohort of 103 adrenocortical tumors from 36 children and 67 adults, whereas gene amplification was studied in 38 adrenocortical tumors (17 from children). METHODS: Tissue microarray, multiplex ligation-dependent probe amplification, and quantitative real-time PCR were used. RESULTS: A strong nuclear SF-1 expression was detected by tissue microarray in 56% (20 of 36) and 19% (13 of 67) of the pediatric and adult adrenocortical tumors, respectively (P = 0.0004). Increased SF-1 copy number was identified in 47% (eight of 17) and 10% (two of 21) of the pediatric and adult adrenocortical tumors, respectively (P = 0.02). All adrenocortical tumors with SF-1 gene amplification showed a strong SF-1 staining, whereas most of the tumors (61%) without SF-1 amplification displayed a weak or negative staining (P = 0.0008). Interestingly, a strong SF-1 staining was identified in five (29%) pediatric adrenocortical tumors without SF-1 amplification. The frequency of SF-1 overexpression and gene amplification was similar in adrenocortical adenomas and carcinomas. CONCLUSION: We demonstrated a higher frequency of SF-1 overexpression and gene amplification in pediatric than in adult adrenocortical tumors, suggesting an important role of SF-1 in pediatric adrenocortical tumorigenesis.
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Authors | Madson Q Almeida, Ibere Cauduro Soares, Tamaya C Ribeiro, Maria Candida B V Fragoso, Lidiane V Marins, Alda Wakamatsu, Rodrigo A Ressio, Mirian Y Nishi, Alexander A L Jorge, Antonio M Lerario, Venancio A F Alves, Berenice B Mendonca, Ana Claudia Latronico |
Journal | The Journal of clinical endocrinology and metabolism
(J Clin Endocrinol Metab)
Vol. 95
Issue 3
Pg. 1458-62
(Mar 2010)
ISSN: 1945-7197 [Electronic] United States |
PMID | 20080844
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
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Topics |
- Adrenal Cortex Neoplasms
(genetics)
- Adrenocortical Adenoma
(genetics)
- Adrenocortical Carcinoma
(genetics)
- Adult
- Age Factors
- Chi-Square Distribution
- Child
- Child, Preschool
- Female
- Gene Amplification
(genetics)
- Gene Expression Regulation, Neoplastic
- Humans
- Male
- Reverse Transcriptase Polymerase Chain Reaction
- Steroidogenic Factor 1
(genetics)
- Tissue Array Analysis
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