(1.12) Resection of symptomatic unilateral glucocorticoid-producing adrenal adenoma

Most adrenocortical adenomas are clinically silent and are usually incidental findings during abdominal imaging not performed for suspected adrenal disease (adrenal incidentaloma), but a few can present with signs and/or symptoms of hormonal excess. Determination of functionality is based on clinical and laboratory evaluation.

Case(s) Description :

A 43-year-old man with past medical history of hypertension diagnosed 10 years ago came to our clinic for reassessment of an adrenal adenoma. Four months ago, he went to his family medicine physician due to refractory hypertension and was referred to a nephrologist due to concerns that it could be due to a secondary cause related to the kidneys. Laboratories were remarkable for elevated glucose levels (119 mg/dL), borderline high triglycerides (193 mg/dL), hypernatremia (148 mEq/L), hypokalemia (3.0 mmol/L), aldosterone, and urine metanephrines within normal limits. Head computed tomography (CT) was unremarkable. Abdominal CT scan was notable for a 2.9 cm left adrenal adenoma; renal doppler ultrasound was unremarkable. He was referred to an endocrinologist; new laboratories were significant for increased 24-hour urinary free cortisol (1293.6 mcg/24 hours, reference range 12.9-253 mcg/24 hours), morning cortisol (43.3 mcg/dL, reference range 4.3-22.4 mcg/dL), afternoon cortisol (29.8 mcg/dL, reference range 3.09-16.66 mcg/dL), and ACTH within normal limits. He then came to our clinic; review of systems was positive for progressive increase in cheek size and chronic back pain; physical examination was unremarkable, other cushingoid features were absent. We referred him for surgical consultation and underwent left sided adrenalectomy; pathology examination showed an adrenocortical adenoma measuring 3.1 x 2.9 x 2.3 cm. One day after surgery he presented severe back pain, magnetic resonance imaging of the spine showed compression vertebral fractures on T9, L1, L3, L5 and chronic degenerative changes associated with hypercortisolism; he underwent vertebroplasty. Hip and spine bone density scan (DXA) indicated osteoporosis. Treatment with denosumab 60 mg every 6 months was started. Cortisol levels began to trend down; a two-day, 2 mg test was performed, and showed adequate suppression; two months later after surgery, cortisol levels were within normal limits. He continued to have hypertension but controlled with treatment. The last follow-up was 11 months later, laboratory studies were within normal limits; hip DXA showed improvement indicating osteopenia. He is currently clinically improving, asymptomatic, and under regular follow-up.

Discussion :

In this case, different from the most common presentation of an adrenocortical adenoma, the incidentaloma, the adrenocortical adenoma manifested signs and symptoms due to hypercortisolism. It is very important to consider adrenocortical adenoma as a cause of refractory hypertension and as a cause of bone disorders. Of note, no glucocorticoid replacement therapy was performed. Clinically, he did not have overt Cushing's syndrome, but he was not compatible with subclinical Cushing's syndrome, which is primarily associated with incidentalomas.