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

Objective: Adrenocortical tumors (ACT) account for no more than 0.2% of the causes of androgen excess (AE). 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: A total of 44 consecutive female patients with ACT-AS and it comparison group of 102 women With non-tumor causes of AE (NTAE). Results: Patients with ACT-AS were older than tire ones with NTAE(37.7 vs 24.8 vears: P<0.001) and I prevalence or hirsutism. acne. and oligo/amenorrhea were not different. Free testosterone was the most commonly, elevated androgen ill ACT-AS (94%). followed by androstenedione (90%). DHEAS and total testosterone (76%). and all three 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 three androgens were elevated in only, 22% of the cases. Free testosterone values above 6.85 pg/ml (23.0 pmol/l) had the best diagnostic value for ACT-AS (sensitivity 82%, confidence interval (CI): 57-96%: specificity 97%. CI: 91-100%. Basal LH and FSH levels were significantly lower in the ACT-AS group. Conclusion: Free testosterone was the most reliable marker of ACT-AS. However. the large overlap or androgen levels between ACT-AS and NTAE groups suggests I hat additional hormonal and/or imaging investigations are required to rule out ACT-AS in case of increased androgens.