CASE REPORT
eISSN 2384-0293 http://dx.doi.org/10.12701/yujm.2015.32.2.132
Yeungnam Univ J Med 2015;32(2):132-137
Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma presented as Cushing syndrome and complicated by invasive aspergillosis Jae Ho Cho1, Da Eun Jeong1, Jae Young Lee1, Jong Geol Jang1, Jun Sung Moon1, Mi Jin Kim2, 1 1 1 Ji Sung Yoon , Kyu Chang Won , Hyoung Woo Lee Departments of 1Internal Medicine and 2Pathology, College of Medicine, Yeungnam University, Daegu, Korea 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. Keywords: ACTH syndrome; Ectopic; Cushing syndrome; Pheochromocytoma; Aspergillosis
INTRODUCTION Ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome comprises approximately 10% of Cushing syndrome [1]. The most common causes of ectopic ACTH syndrome are malignancies that include small cell lung cancer (SCLC), bronchial carcinoids, islet cell tumors of the pancreas, thymic carcinoids, and medullary thyroid cancer. ACTH-producing pheochromocytoma is one cause of ectopic ACTH syndrome, which has been reported to range from 2% to 25% [2-8], fewer than 30 cases globally and only 3 cases have been reported in Korea [9-12]. Received: July 24, 2014, Revised: September 12, 2014, Accepted: September 15, 2014 Corresponding Author: Hyoung Woo Lee, Department of Internal Medicine, College of Medicine, Yeungnam University, 170 Hyeonchung-ro, Namgu, Daegu 42415, Korea Tel: +82-53-620-3839, Fax: +82-53-623-8006 E-mail:
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Endogenous and iatrogenic hypercortisolism is known to impair cell-mediated immunity, making patients with Cushing syndrome susceptible to opportunistic infections such as mucocutaneous and invasive fungal infections, viral infections, bacterial infections, reactivation of tuberculosis, and post-operative wound infections [13,14]. However, invasive aspergillosis is rarely reported in patients with Cushing syndrome. This case is the first report of invasive aspergillosis complicated by ACTH-producing pheochromocytoma.
CASE A 35-year-old woman was admitted to our hospital due to headache for 2 months. She also suffered from proximal muscle weakness and ocular pain. She had not had any other disease, but 2 months before her admission, she was diagnosed with hypertension and treated with 3 kinds of antihypertensive drugs (an α1-receptor blocker, a calcium antag-
YUJM VOLUME 32, NUMBER 2, DECEMBER 2015
ACTH-producing pheochromocytoma complicated by invasive aspergillosis
phrine (44.3 μg/day; NR, 0-20 μg/day), norepinephrine (121.6 μg/day; NR, 15-80 μg/day), and metanephrine (1.8 mg/day; NR, 49 After low-dose suppression >49 After high-dose suppression >49 Serum ACTH (pg/mL) Basal (8:00 a.m.) 10-60 357.89 47 Basal (4:00 p.m.) 6-30 289.54 Serum epinephrine (pg/mL) 5,000 After high-dose suppression 5,565 24-hr urine epinephrine (μg/day) 0-20 44.3 24-hr urine norepinephrine (μg/day) 15-80 121.6 24-hr urine VMA (mg/day) 0-8 2.6 24-hr urine metanephrine (mg/day)
