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This Article Accepted manuscript (PDF) All Versions of this Article: EJE-08-0324v1 159/5/641    most recent Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Google Scholar Articles by d'Alva, C. Articles by Bertherat, J. PubMed PubMed Citation Articles by d'Alva, C. Articles by Bertherat, J.

Sex steroids in androgen-secreting adrenocortical tumors: clinical and hormonal features in comparison with non tumoral causes of androgen excess

C d'Alva, Endocrinology, Hopital Cochin, Paris, France
G Abiven-Lepage, endocrinology, Hopital Cochin, Paris, France
V Viallon, Biostastitics, Hopital Cochin, Paris, France
L Groussin, paris, France
M Dugue, hormonologie, hopital Cochin, paris, France
X Bertagna, paris, France
J Bertherat, Clinique des Maladies Endocriniennes et Mï¿taboliques, Hï¿pital Cochin, Paris, F-75014 , France

Correspondence: Jerome Bertherat, Email: jerome.bertherat{at}cch.aphp.fr

Abstract

Objective: Adrenocortical tumors (ACT) account for no more than 0.2% of the androgen excess (AE) causes. Conversely, these rare tumors have a very poor prognosis. It is difficult and important to exclude this diagnosis whenever there is AE.

Design: Retrospective investigation of androgen profiles in a large consecutive series of androgen secreting (AS) ACT to assess their relative diagnostic value.

Methods: 44 consecutive female patients with ACT-AS and a comparison group of 102 women with non tumor causes of AE (NTAE).

Results: Patients with ACT-AS were older than the ones with NTAE (37.7 vs 24.8 yr; p<0.001) and the prevalence of hirsutism, acne and oligo/amenorrhea were not different. Free testosterone was the most commonly elevated androgen in ACT-AS (94%), followed by androstenedione (90%), DHEAS (82%) and total testosterone (76%), and all 3 androgens were simultaneously elevated in 56% of the cases. Androgen serum levels became subnormal in all ACT-AS patients after complete tumor removal. In NTAE, the most commonly elevated androgen was androstenedione (93%), while all 3 androgens were elevated in only 22% of the cases. Free testosterone values above 6.85 pg/mL (23.6 pmol/L) had the best diagnostic value for ACT-AS (sensitivity 82%, CI: 57-96%; specificity 97%, CI: 91-100%). Basal LH and FSH levels were significantly lower in ACT-AS group.

Conclusion: Free testosterone was the most reliable marker of ACT-AS. However, the large overlap of androgen levels between ACT-AS and NTAE groups suggests that additional hormonal and/or imaging investigations are required to rule out ACT-AS in case of increased androgens.