Pregnancy Does Not Accelerate Corticotroph Tumor Progression in Nelson's Syndrome

Abstract

Pituitary surgery is the first line of treatment for Cushing's disease; when surgery fails, bilateral adrenalectomy may be proposed, particularly for women with a desire for pregnancy. Little is known about the impact of pregnancy on corticotroph tumor progression after bilateral adrenalectomy. The aim was to evaluate the impact of pregnancy on corticotroph tumor progression after bilateral adrenalectomy in Cushing's disease and to assess maternal and pregnancy outcomes. We conducted a retrospective cohort study. Patients who became pregnant after bilateral adrenalectomy were followed in a single center. Twenty pregnancies from 11 patients with Cushing's disease were treated by bilateral adrenalectomy and no pituitary irradiation. Corticotroph tumor progression was assessed by serial pituitary magnetic resonance imaging and plasma ACTH measurements before, during, and after pregnancy. Comparisons were performed using paired Wilcoxon rank tests. Data on maternal and neonatal outcomes were recorded by correspondence from patients and obstetricians. Corticotroph tumor progression occurred in eight of 17 pregnancies, and ACTH increased in eight of 10 pregnancies. However, rates of increase during or after pregnancy were not faster than those observed before pregnancy. Maternal complications occurred in four pregnancies from two patients, including gestational hypertension in three and gestational diabetes mellitus in three. Among these four pregnancies, three had a favorable outcome, and one led to an in utero death after eclampsia, due to loss to follow-up. No other maternal or fetal complications were reported. Pregnancy does not accelerate corticotroph tumor progression after bilateral adrenalectomy. Pregnancy is manageable, provided the patients can be followed closely.