Cushing syndrome (CS) in pregnancy is rare with only about 200 cases reported in literature.In pregnant women, 50% of cases is caused by adrenal adenoma. The diagnosis is challenging due to overlapping symptoms with normal pregnancy.

A 29-year-old Filipino woman presented with subtle signs of hypercortisolism: nail hyperpigmentation, bipedal edema and moon facie 3 months prior to pregnancy. However, during pregnancy at 4 weeks age of gestation, her condition progressed and she developed classic clinical features of Cushing syndrome, including facial plethora, cutaneous purple striae, easy bruising, central obesity, weight gain, buffalo hump, acne and mild hirsutism. Notably, she experienced blurring of vision and brown spots on visual field in the left eye. She consulted an ophthalmologist and optical coherence tomography confirmed the diagnosis of central serous retinopathy. She was then referred to an endocrinologist for work up of an endocrine cause.

Initial tests were consistent with ACTH-independent CS: elevated 24-hour urine free cortisol 575.10 µg/24hrs (normal 4.3- 176), unsuppressed 8am cortisol after overnight 1mg low dose dexamethasone suppression test 1,258.1 nmol/L (normal 101.2- 535.7) and low ACTH 10pg/ml (normal <40). MRI of the adrenals showed left adrenal adenoma with benign features measuring 1.9 x 2.7 x 3.0 cm, a co-secreting tumor of the adrenals was ruled out since with unremarkable findings: PAC 2 ng/dL (normal ≤ 28), PRA 3.52 ng/ml/h (normal 0.25- 5.82), ARR 0.6 (normal 0.9- 28.9), plasma free metanephrine <0.08 nmol/L (normal <0.36) and DHEAS 6.7µmol/L (normal 2.6-13.9). She was started on insulin regimen for hyperglycemia. At 17 weeks of gestation, the patient had multiple hospital admissions for hypokalemia and elevated blood pressure. She was managed with oral potassium supplementation and methyldopa. Given the severity of symptoms and optimal timing of surgery in the second trimester, she underwent a successful laparoscopic left adrenalectomy. Postoperative 8am serum cortisol dropped to 5.50 µg/dL (normal 3.7–19.4). Histopathology confirmed an adrenocortical adenoma. She was discharged on hydrocortisone replacement therapy. One month after surgery, she showed marked clinical improvement, with resolution of hypertension and hypokalemia and improved glycemic control.

Early recognition and management of CS in pregnancy is crucial. Laparoscopic adrenalectomy is safe and effective treatment for cortisol-secreting adrenal adenomas.