Adrenocorticotropic hormone-independent Cushing syndrome manifesting during pregnancy

To report a case of adrenocorticotropic hormone-independent Cushing syndrome (CS) diagnosed and treated surgically during the third trimester of pregnancy and resulting in delivery of a healthy baby boy.

We present a detailed case report, and we review and evaluate the English-language literature on CS during pregnancy.

During pregnancy, the occurrence of CS is a rare event. The diagnosis of CS during pregnancy is difficult to establish because of the normal physiologic hypercortisolemia of pregnancy. In our patient, laboratory testing revealed a random serum cortisol level of 56.5 microg/dL, a suppressed plasma adrenocorticotropic hormone level (<5 pg/mL), and a substantially elevated 24-hour urinary cortisol (1,708 microg). Noncontrast magnetic resonance imaging of the abdomen disclosed a 3.5-cm left adrenal mass. Laparoscopic left adrenalectomy was successfully performed during the early third trimester, and a healthy baby was born at 36 weeks of gestation by means of a cesarean delivery.

The occurrence of CS during pregnancy is rare; however, when it does occur, adrenal tumors are more common than pituitary tumors. Caution should be used during interpretation of laboratory tests to evaluate for CS during pregnancy because of the normal increase in hypothalamic-pituitary-adrenal axis function during pregnancy. The current case demonstrates the safety and utility of noncontrast magnetic resonance imaging for localization of a tumor during pregnancy, as well as the safe use of laparoscopic surgical treatment of CS during the early third trimester.