Endocrine Abstracts

Introduction: Pheochromocytoma is a functional catecholamine producing tumor that originates from adrenal chromaffin cells and is an extremely rare cause of ectopic Cushing syndrome. We report clinical case of adrenal incidentaloma with the evidence of both pheochromocytoma and sub-clinical Cushing’s syndrome.

Observation: A fifty-two year-old female patient was referred to our institution because of an incidentally discovered right adrenal mass. Although she had mild hypertension, she had no signs of excessive production of either catecholamines or adrenocortical steroids. Abdominal computed tomography revealed right adrenal solid mass with the diameter of 6 cm. The tumor was visualized as a heterogeneous mass with a central area of necrosis. Left adrenal gland was normal. The laboratory data demonstrated three times raised twenty four hours urinary fractionated metanephrines with non-suppressible serum cortisol after two-day low-dose dexamethasone suppression test. Her basal corticotrophin concentration was nineteen pg/ml. Pituitary magnetic resonance imaging (MRI) did not reveal any lesion. After pre-operative management with alpha blocker, she underwent laparoscopic right adrenalectomy. The pathologist concluded that the lesion was pheochromocytoma . The patient developed adrenal insufficiency after surgery and required glucocorticoid replacement therapy for eight months.

Discussion: Patients with ACTH-secreting pheochromocytoma are seen in five per cent of patients with the ectopic Cushing syndrome, and pose distinctive diagnostic and management challenges, but if diagnosed early and managed intensively they should be curable by surgery.

Conclusion: This case represents a very rare cause of ectopic Cushing syndrome caused by an ACTH producing pheochromocytoma. Extensive hormone profile evaluation should be carried out in all adult patients presenting with an adrenal mass independent of their clinical presentation.