Adrenocortical carcinoma and pregnancy: clinical and biological features and prognosis.
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.


METHODS
Clinical presentation, hormonal secretion, staging, survival, and obstetric data are reported. Patients were included between 1963 and 2007. Mean follow-up was 48 months.


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 (n=110). 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 (P=0.06) 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 interval=1.34-11.85, P=0.013).


CONCLUSIONS
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.
Abiven-Lepage
Gwenaëlle
G
Department of Endocrinology, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.
Coste
Joël
J
Tissier
Frédérique
F
Groussin
Lionel
L
Billaud
Line
L
Dousset
Bertrand
B
Goffinet
François
F
Bertagna
Xavier
X
Bertherat
Jérôme
J
Raffin-Sanson
Marie-Laure
ML
D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D018268:Adrenocortical Carcinoma / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D000328:Adult; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008875:Middle Aged; D049590:Postpartum Period / Q000502:physiology; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D047928:Premature Birth / Q000175:diagnosis / Q000209:etiology; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult