Yeungnam Univ J Med.  2015 Dec;32(2):132-137. 10.12701/yujm.2015.32.2.132.

Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma presented as Cushing syndrome and complicated by invasive aspergillosis

• Cho JH
• Jeong DE
• Lee JY
• Jang JG
• Moon JS
• Kim MJ
• Yoon JS
• Won KC
• Lee HW

• KMID: 2319915
• DOI: http://doi.org/10.12701/yujm.2015.32.2.132

Abstract

Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma has been rarely reported, whereas only a few cases of Cushing syndrome accompanied by opportunistic infections have been reported. We experienced a patient with pheochromocytoma with ectopic Cushing syndrome complicated by invasive aspergillosis. A 35-year-old woman presented with typical Cushingoid features. Her basal plasma cortisol, ACTH, and 24-hour urine free cortisol levels were significantly high, and 24-hour urine metanephrine and catecholamine levels were slightly elevated. The endogeneous cortisol secretion was not suppressed by either low- or high-dose dexamethasone. Abdominal computed tomography (CT) revealed a heterogeneous enhancing mass measuring approximately 2.5 cm in size in the left adrenal gland. No definitive mass lesion was observed on sellar magnetic resonance imaging. On fluorine-18 fluorodeoxyglucose positron emission tomography/CT, a hypermetabolic nodule was observed in the left upper lung. Thus, we performed a percutaneous needle biopsy, which revealed inflammation, not malignancy. Thereafter, we performed a laparoscopic left adrenalectomy, and its pathologic finding was a pheochromocytoma with positive immunohistostaining for ACTH. After surgery, the biochemistry was normalized, but the clinical course was fatal despite intensive care because of the invasive aspergillosis that included the lungs, retina, and central nervous system.

Keyword

ACTH syndrome; Ectopic; Cushing syndrome; Pheochromocytoma; Aspergillosis

MeSH Terms

Adrenal Glands
Adrenalectomy
Adrenocorticotropic Hormone*
Adult
Aspergillosis*
Biochemistry
Biopsy, Needle
Central Nervous System
Cushing Syndrome*
Dexamethasone
Electrons
Female
Humans
Hydrocortisone
Inflammation
Critical Care
Lung
Magnetic Resonance Imaging
Metanephrine
Opportunistic Infections
Pheochromocytoma*
Plasma
Retina
Adrenocorticotropic Hormone
Dexamethasone
Hydrocortisone
Metanephrine