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Neuroendocrine and Pituitary
Tumor Clinical Center (NEPTCC) Bulletin

Winter 2017/2018 | Volume 24, Issue 1

Cushing’s Disease in Pregnancy

Melanie Schorr, M.D.

Case presentation

A 28-year-old woman was referred to the Neuroendocrine and Pituitary Tumor Clinical Center for management of Cushing’s disease (CD). Four years prior to referral, she had a full-term uncomplicated pregnancy and delivered a baby girl. Post-partum, she experienced depression, fatigue, hirsutism, and easy bruising, as well as inability to lose weight and secondary amenorrhea. Three years prior to referral, an outside endocrinologist suspected Cushing’s syndrome (CS), and checked 24-hour urine free cortisols (UFC), which were 10x the upper limit of normal (ULN), and late night salivary cortisols (LNSC), which were also elevated. Serum adrenocorticotropic hormone (ACTH) was elevated, suggesting ACTH-dependent CS. A pituitary MRI revealed a 12 x 9 x 6 mm right sellar lesion. The patient then underwent transsphenoidal surgery at an outside hospital, and pathology was consistent with an ACTH-producing pituitary adenoma. Post-operatively, UFC normalized, but she did not become adrenally insufficient, suggesting she was not in remission and was at a high risk of recurrent hypercortisolemia. Post-operative MRI did not show clear residual tumor.

Symptomatically, the patient felt much improved; her mood, fatigue, hirsutism and easy bruising improved, she was able to lose 20 lbs and had resumption of her menstrual periods. However, two years later, symptoms recurred and she gained 100 lbs. She was referred back to her outside endocrinologist, who suspected persistent CD, and checked UFC, which were 1.5x ULN, and LNSC, which were also elevated. Pituitary MRI demonstrated a small area of residual/recurrent disease in the medial wall of the right cavernous sinus. The patient was then referred to the Neuroendocrine and Pituitary Tumor Clinical Center for management of persistent CD. On exam, she did not appear particularly Cushingoid, as she had normal fat distribution, no extremity thinning and no posterior cervical or supraclavicular fat pads. She did have light violaceous striae over her abdomen. It was recommended that more UFC and LNSC be collected to determine the degree of cortisol excess. Plan was also made for referral to an expert pituitary surgeon, but in the interim, the patient called to report that she was pregnant. Cortisol testing, which had been performed when she was at 8 weeks gestation prior to knowledge of the pregnancy, revealed UFC that were approximately 4x ULN and elevated LNSC. This case raised a number of questions, namely (1) How do you interpret UFC and LNSC in pregnancy? (2) Does CS during pregnancy adversely affect maternal and fetal outcomes? (3) Does treatment of CS during pregnancy improve maternal and fetal outcomes? and (4) What are the treatment options for CS during pregnancy?

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