Endocrine Abstracts (2009) 20 P672

ACTH-independent Cushing's syndrome in pregnancy with normal adrenal glands: ectopic receptors to chorionic gonadotrophin hormone (HCG)?

Baudoux Florence, Devemy Fabrice, Leroy Clara, Valat Anne-Sophie, Faivre-Defrance Frederique & Vantyghem Marie-Christine

Lille 2 University, Lille, France.

Cushing’s syndrome during pregnancy is a rare disorder. A 32-year-old pregnant woman presented with Cushing’s syndrome. She had had a medical abortion complicated with bleeding for anencephaly by 15 weeks of her first pregnancy. At the beginning of her second pregnancy, typical clinical signs of Cushing syndrome developed: extensive red stretch marks on the abdomen and thighs, excessive weight gain (24 kg over 17 weeks) and high blood pressure (150/90 mmHg). By week 22 of gestation, plasma cortisol diurnal rhythm was absent (Midnight cortisol level: 40 μg/dl;N:3–7 μg/dl) and there was a paradoxical increase in plasma cortisol after a 1-mg dexamethasone overnight suppression test (30 μg/dl; Normal <1.8). Basal urinary free cortisol was 3 fold above the upper limit and ACTH levels were suppressed. Blood HCG levels were 14 785 UI/l. The diagnosis of ACTH-independent Cushing’s syndrome was established. MRI scans revealed normal adrenal and pituitary glands. Spuriously, by 26 weeks of gestation, clinical signs slightly dwindled with a 2 kg weight loss whereas blood HCG levels decreased to 10 840 UI/l. As hypertension was controled with labetalol and nicardipine, and gestational diabetes with diet, no further treatment with metyrapone was proposed. By week 28, signs of preeclempsia (high blood pressure, moderate oedema, platelets fall, increase of liver enzyme levels) led to perform a caesarean section (CS). The newborn was a normal virilized male who weighed 1490 g. The mechanisms by which pregnancy-induced Cushing’s syndrome occurred in this patient are unclear. Aberrant responsiveness or hyperresponsiveness of adrenocortical cells to a non-ACTH and non-CRH substance produced in excess in pregnancy should be considered. Ectopic adrenal receptor for HCG are suspected. Nevertheless the last assessment 1 week after CS showed the persistence of ACTH-independent Cushing’s syndrome leading to suspect a Carney complex. A genetic study is in progress.

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