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Endocrine Abstracts (2014) 35 P6 | DOI: 10.1530/endoabs.35.P6

ECE2014 Poster Presentations Adrenal cortex (56 abstracts)

An autopsy case of ectopic ACTH-secreting lung carcinoid with Cushing's syndrome

Yui Watanabe 1 , Keiichiro Matoba 1, , Hiroyuki Yamazaki 2 , Katsuyoshi Tojo 1 & Kazunori Utsunomiya 1


1Division of Diabetes, Metabolism and Endocrinology, The Jikei University School of Medicine, Tokyo, Japan; 2Division of Diabetes and Endocrinology, Department Kawaguchi Municipal Medical Center, Saitama, Japan.


A 81-year-old woman came to our hospital with complaints of reduction in appetite and weakness of lower limbs. She had developed Cushing’s features. Initial laboratory evaluation revealed severe hypokalemia and hyperglycemia without ketoacidosis, and diabetes mellitus had not been detected previously. A series of hormonal assessment revealed marked ACTH-dependent hypercortisolism. Corticotrophin-releasing hormone stimulation test resulted in normal ACTH response, thus we ran additional tests to make a diagnosis of Cushing’s disease. However, magnetic resonance imaging showed no obvious tumor in her hypophysis. Furthermore, whole-body computed tomography and positron emission computed tomography did not identify apparent tumor. Therefore, we diagnosed it as occult ectopic ACTH-secreting tumor, so we administered octreotide acetate 20 mg/4 weeks and trilostane 240 mg/day. After this treatment, hormonal level had been slightly improved. However, she had been compromised and had experienced recurrent infections, for example, pneumonia and infectious spondylitis. Finally, she was admitted to our department for inflammation of the bile duct. She developed septic shock and dis-seminated intravascular coagulation. Although we started antibiotic, vasopressor and lyophilized human antithrombin? concentrate, she deteriorated rapidly and died 2 day later. Autopsy was performed and showed a solitary adrenal corticotropic hormone-producing neuroendocrine tumor in the right lung, which was 17 mm in diameter. This tumor was stained by CD56, chromogranin a and synaptophysin, so we diagnosed ectopic adrenocorticotropic hormone secreting lung carcinoid. We experienced a case that we could not detect a localization of ACTH-secreting tumor regardless of repeated radiological imaging, but autopsy confirmed its diagnosis.

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