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MON-103 Successful Medical Management of Cushing Disease With Metyrapone Throughout Pregnancy: A Report of Two Cases

Year: 2025
Type: article
Abstract: Disclosure: A. Theodorou: None. E.B. Geer: Serving as an investigator for research grants to MSKCC from Recordati, Sparrow, and Xeris. Occasional consultant to HRA Pharma, Sparrow, Xeris, and Lundbeck. Background: Cushing syndrome (CS) in pregnancy is a rare disorder with challenging diagnostic and therapeutic considerations. For patients with medically managed CS planning pregnancy, bilateral adrenalectomy (BLA) is advised before conception. No pharmacologic treatment is FDA-approved for use in pregnancy. To our knowledge, no case of Cushing Disease (CD) medically treated throughout the entire gestation with cortisol synthesis inhibition has been reported. We describe, for the first time, two patients with CD, treated successfully pre-conception and throughout pregnancy with the cortisol synthesis inhibitor metyrapone. Clinical cases: Case 1: A 31-year-old woman with post-operative recurrence of CD expressed a desire to conceive but declined BLA. She proceeded with in vitro fertilization while on metyrapone, 500 mg three times daily. Her pregnancy was closely monitored, with ongoing assessments for gestational diabetes and hypertension. Biochemical evaluation including measurements of plasma ACTH, serum cortisol, 24-hr urine free cortisol (UFC), and late night salivary cortisol (LNSC) values, was performed every 4 weeks, and metyrapone dose was adjusted based on biochemical results. She had an uneventful pregnancy, delivering healthy twins at 37 weeks, while on metyrapone, via cesarean section. Postpartum, she developed hypertension, which resolved spontaneously, and remained on metyrapone at 250 mg in the afternoon and 500 mg at bedtime, with stable cortisol levels and normal development of her twins. Case 2: A 29-year-old woman with recurrent CD since adolescence, status post two transsphenoidal resections, and gamma knife radiosurgery, conceived naturally on metyrapone 250mg four times daily. Metyrapone dose was maintained at a stable dose throughout pregnancy, with close monitoring of her 24-hour UFC levels. The patient opted to stop metyrapone before cesarean section and resumed four days postpartum. After an uncomplicated pregnancy, she delivered a healthy baby girl, without evidence of adrenal insufficiency. One week after delivery, she developed postpartum pre-eclampsia, requiring temporary antihypertensive therapy, including labetalol, nifedipine, and magnesium for three weeks. Follow-up demonstrated well-controlled cortisol levels; her child exhibited normal growth and development at five years. Clinical Lesson/ Conclusion: While not FDA-approved for pregnancy, cortisol synthesis inhibition with metyrapone may be a viable therapeutic option for managing CD during pregnancy when surgical intervention is contraindicated or declined. Careful monitoring, multidisciplinary management, and dose optimization are essential. Particular attention is needed for hypertensive complications, even in the post-partum period. Further investigation is required to establish safety and optimal dosing strategies. Presentation: Monday, July 14, 2025
DOI: 10.1210/jendso/bvaf149.1432
Language: English
Field: Medicine
Open Access status: gold
Estimated APC paid: $3,255
OpenAlex ID: W7093359002
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