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.


METHODS
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 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 years; 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 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 (23.6 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.


CONCLUSIONS
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.
d'Alva
Catarina B
CB
Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Service d'Endocrinologie, Paris, France.
Abiven-Lepage
Gwenaelle
G
Viallon
Vivian
V
Groussin
Lionel
L
Dugue
Marie Annick
MA
Bertagna
Xavier
X
Bertherat
Jerôme
J
D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000378:metabolism*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000728:Androgens / Q000097:blood* / Q000378:metabolism*; D000735:Androstenedione / Q000097:blood / Q000378:metabolism; D014408:Biomarkers, Tumor / Q000097:blood*; D019314:Dehydroepiandrosterone Sulfate / Q000097:blood; D004967:Estrogens / Q000097:blood / Q000378:metabolism; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D006801:Humans; D006854:Hydrocortisone / Q000097:blood / Q000378:metabolism; D007986:Luteinizing Hormone / Q000097:blood; D008875:Middle Aged; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D013739:Testosterone / Q000097:blood / Q000378:metabolism
D000728:Androgens; D014408:Biomarkers, Tumor; D004967:Estrogens; D013739:Testosterone; D000735:Androstenedione; D019314:Dehydroepiandrosterone Sulfate; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D006854:Hydrocortisone