Objective: Adrenocortical carcinoma (ACC) is a rare, severe disease. Pregnancy-associated ACC has rarely been reported. We wished to evaluate the characteristics and prognosis of ACC diagnosed in patients during pregnancy or in the postpartum period, comparing them with those for ACC diagnosed in nonpregnant women. Design: Clinical presentation, hormonal secretion, staging, survival, and obstetric data are reported. Patients were included between 1963 and 2007. Mean follow-up was 48 months. Patients and methods: This is a retrospective cohort study carried out at a referral center. All female patients aged 16-49 years diagnosed with ACC during the observation period were included (nZ110). Twelve of these women were pregnant or in the first 6 months after delivery. Hormonal secretion, staging, obstetric data, and survival were analyzed. For the survival analysis, pregnant patients were compared with a subgroup of nonpregnant women matched for age, stage, and year of diagnosis (1 pregnant patient/2 controls). Results: Adrenocortical tumors diagnosed during pregnancy or in the postpartum period tend to be more often cortisol-secreting tumors (PZ0.06) and to be discovered at a more advanced stage than those in nonpregnant women, although the differences were not significant. Fetal outcome was poor. Overall survival of the mother was worse than that of matched controls (hazard ratio of death: 3.98, confidence intervalZ1.34-11.85, PZ0.013). Conclusion: ACC diagnosed during pregnancy or in the postpartum period is associated with a poor fetal outcome and a poorer prognosis than ACC diagnosed in nonpregnant women.

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 androgensecreting (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. 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.European Journal of Endocrinology 159 641-647