Adrenocortical carcinoma and pregnancy: clinical and biological features and prognosis.
Journal - European journal of endocrinology / European Federation of Endocrine Societies
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 during pregnancy or in the post partum period, comparing them with those for ACC diagnosed in non pregnant 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 to 49 years diagnosed with ACC during the observation period were included (n=110). Twelve of these women were pregnant or in the first six months after delivery. Hormonal secretion, staging, obstetric data and survival were analyzed. For the survival analysis, pregnant patients were compared with a subgroup of non pregnant women matched for age, stage, and year of diagnosis (1 pregnant patient/2 controls). Results: Adrenocortical tumors diagnosed during pregnancy or in the post partum period tend to be more often cortisol-secreting tumors (p=0.06) and to be discovered at a more advanced stage than those in non pregnant 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 interval = 1.34-11.85, p=0.013) Conclusion: ACC diagnosed during pregnancy or in the post partum period is associated with a poor fetal outcome and a poorer prognosis than ACC diagnosed in non pregnant women.