Endocrine Abstracts

Introduction: ACTH-producing pheochromocytoma has been rarely reported, ranging from 3 to 25% of the ectopic ACTH syndrome. Moreover, a few cases of Cushing’s syndrome (CS) accompanied with opportunistic infections have been reported. We experienced a case of pheochromocytoma with ectopic CS, complicated by invasive aspergillosis.

Case report: A 35-year-old woman visited our hospital because of headache for 2 months. She presented with typical cushingoid features including centripetal obesity, buffalo hump, moon face, and hirsutism. Basal plasma cortisol and ACTH, and 24 h-urine free cortisol levels were significantly high while urinary metanephrine and catecholamine levels (24 h) were slightly elevated. The endogeneous cortisol secretion was not suppressed by both low and high dose dexamethasone. On abdominal computed tomography (CT), about 2.5 cm sized heterogeneous enhancing mass on the left adrenal gland was found. There was no definitive mass lesion on sellar magnetic resonance imaging. On PET-CT, a hypermetabolic nodule was found in left upper lung. So, we performed percutaneous needle biopsy, which was revealed inflammation, not malignancy. Thereafter, we performed left adrenalectomy, and it’s pathologic finding was a pheochromocytoma with positive immunohistostaining for ACTH. After surgery, biochemistry was normalized but the clinical course was fatal despite of intensive care because of invasive aspergillosis included lung, retina, and CNS.

Conclusions: If the patient who has adrenal mass showed both catecholamine and corticosteroid excess, ‘ACTH-producing pheochromocytoma’ should be considered as one of differential diagnosis. Meanwhile, high cortisol level is generally observed in patients with ectopic ACTH CS, it may result in fatal outcomes because susceptible to opportunistic infections. Thus, careful attention and prompt management are needed to treat these patients.