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Steroidogenic factor 1 overexpression and gene amplification are more frequent in adrenocortical tumors from children than from adults.

AbstractBACKGROUND:
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.
AuthorsMadson 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
JournalThe 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
PMID20080844 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Steroidogenic Factor 1
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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