SUCCESSFUL PREGNANCY OF A PATIENT WITH CUSHING'S SYNDROME AFTER REMOVAL OF AN ADRENAL MACROADENOMA ASSOCIATED WITH AN ACTH SECRETING PITUITARY ADENOMA

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 1-36-Adrenal Incidentaloma & Carcinoma
Clinical
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-14
Carla Maria Romero1, Halis Sonmez*2, Amit Kumar Seth3 and Agustin Busta4
1Beth Israel Medical Center, New York, NY, 2George Washington University, Washington, DC, 3Saint Michael's Medical Center, Newark, NJ, 4Beth Israel, New York, NY
Introduction: We report resolution of symptoms and a successful pregnancy after adrenalectomy in a patient with hypercortisolism

Case: 22 yo woman presented with galactorrhea and amenorrhea since age 16, 20 lb weight gain over 1 year, lower extremity edema, hirsutism, and a recent miscarriage. Exam showed obesity, moon facies, acne, buffalo hump, supraclavicular fat pad, bilateral galactorrhea, and abdominal purple striae. Visual field exam was normal. 24 hour urine showed cortisol 513.2 mcg (nl 23-195), 17-ketosteroids 28.3 mg (nl 6-15), 17-OH-corticosteroids 25.1 mg (nl 2-6), and normal metanephrines. DHEA-S was 586 mcg/dl (nl 45-320), prolactin 21.7 ng/ml (nl 2.8 – 29.2). After 8 mg overnight dexamethasone suppression test, cortisol 22.7 mcg/dl, ACTH 11 pg/ml, DHEA-S 756 mcg/dl. Abdominal CT showed a 5.4 x 3.8 cm right adrenal mass consistent with adrenal adenoma. Brain MRI revealed diffuse enlargement of the pituitary gland extending into the suprasellar cistern with slight compression of the optic chiasm. She underwent right adrenalectomy, pathology showed a 5.5 cm adrenal cortical adenoma. She became pregnant 2 months after surgery. She was tapered off steroids, she has lost 22 lbs and her symptoms resolved.

Clinical lessons: Coexistence of unilateral adrenal Cushing’s and pituitary adenoma is rare. There are conflicting reports in the literature regarding both its pathophysiology and incidence. There is a hypothesis of a hyperplasia-adenoma sequence, with a transition from pituitary-dependent to adrenal adenoma-dependent Cushing’s syndrome. The patient’s cortisol did not suppress with 8 mg dexamethasone, which is seen in ACTH-independent hypercortisolism; however ACTH and DHEA-S did not suppress, as seen in ACTH–dependent hypercortisolism. With this conflicting data, in the presence of a large adrenal mass, she underwent adrenalectomy. Her symptoms and physical stigmata have since resolved. She does not require exogenous steroids, and had a successful pregnancy. This indicates her hypercortisolism originated from the adrenal adenoma; however, her enlarged pituitary, mild increase in ACTH, and increased DHEA-S, point to the possible coexistence of pituitary disease. Hence, we need to continue monitoring her pituitary, with ACTH and cortisol levels.

The management of coexistent unilateral adrenal Cushing’s and a probable pituitary adenoma is unclear and controversial. It requires interdisciplinary workup and management, with close follow-up.

Nothing to Disclose: CMR, HS, AKS, AB

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