Cushing's Syndrome in Women With Polycystic Ovaries and Hyperandrogenism

P. Gerry Fegan; Derek D. Sandeman; Nils Krone; Deborah Bosman; Peter J. Wood; Paul M. Stewart; Neil A. Hanley

Disclosures

Nat Clin Pract Endocrinol Metab. 2007;3(11):778-783. 

In This Article

The case

A 41-year-old woman presented at an endocrinology-gynecology clinic. Seven years previously she had tbeen investigated at another hospital for primary infertility of 5 years standing, associated with secondary amenorrhea for the preceding 6 months, oily skin, acne and facial hirsutism. At the time, laparoscopy and dye infusion had demonstrated multiple, small ovarian cysts with patent oviducts. A diagnosis of polycystic ovarian syndrome (PCOS) was made. Attempts at ovulation induction were then undertaken with 50 mg clomifene citrate on days 2-6 of the menstrual cycle, following which she conceived aged 35 years and carried a successful pregnancy to term, giving birth to a healthy baby girl.

Subfertility was again problematic and led to the patient's referral for in vitro fertilization, one cycle of which was unsuccessful. At this time, the patient's BMI was 26 kg/m2 and investigations revealed serum total testosterone levels of 2.6 nmol/l (75 ng/dl; normal range 0.5-2.6 nmol/l [14-75 ng/dl]) and serum estradiol levels of 47 pmol/l (13 pg/ml; normal range 100-500 pmol/l [27-136 pg/ml] early follicular phase; 300-1,250 pmol/l [82-341 pg/ml] luteal phase). The patient's serum androstenedione, dehydroepiandrosterone sulfate and gonadotropin levels were normal. She was commenced on metformin off-licence (titrated up to a dose of 500 mg three times daily).[1] Oligomenorrhea, however, persisted and weight gain increased. Hirsutism and acne remained troublesome. The patient's attempts to conceive were abandoned. Dianette® (cyproterone acetate 2 mg plus ethinylestradiol 35 µg; Schering Health, Burgess Hill, UK) was prescribed, but was later withdrawn by the primary care physician because the patient was hypertensive.

Upon examination, the patient's plethoric round facies, purple abdominal striae and supraclavicular fat pads raised concern that she had Cushing's syndrome. There was no bruising or proximal myopathy. Her blood pressure in the clinic on ramipril 2.5 mg and bendroflumethiazide 2.5 mg was 175/105 mmHg. On metformin, random blood glucose was 5.0 mmol/l (90 mg/dl; reference range for fasting blood glucose 3.6-5.5 mmol/l [65-99 mg/dl]) and serum electrolyte levels were normal.

Screening tests were performed for Cushing's syndrome. Three 24-h collections for urinary free cortisol demonstrated 784, 1,187, and 608 nmol per specimen (normal < 260 nmol per specimen), 1 mg of dexamethasone failed to suppress overnight cortisol (537 nmol/l; normal < 50 nmol/l) and a bedtime salivary cortisol test confirmed loss of diurnal variation (22.6, 14.8 and 22.1 nmol/l; reference range from 2200 h to 2400 h < 5 nmol/l). Serum adrenocorticotropic hormone was undetectable. CT of the abdomen showed a 2.5-cm-diameter mass in the right adrenal gland with features of high lipid content (Figure 1A). The patient was diagnosed with Cushing's syndrome secondary to an adrenal adenoma. She underwent laparoscopic right adrenalectomy without complication. Pathological analysis of the removed adrenal gland revealed a lipid-rich tumor consistent with a functional adrenocortical adenoma. Within 10 months, all symptoms had resolved (Figure 1B,C).

Figure 1.

Abdominal CT scan and patient's appearance. (A) Abdominal CT from the patient showing a right adrenocortical adenoma (arrow). The same patient is shown (B) 1 month before and (C) 6 months after laparoscopic right adrenalectomy.

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