A Rare Case of Ectopic Adrenocorticotropic Hormone Syndrome with Recurrent Olfactory Neuroblastoma |
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| Authors: | Hironori Abe Hirotsugu Suwanai Nodoka Kambara Koji Sano Junpei Shikuma Hiroaki Akaoka Akira Kanazawa Hideaki Hirai Takashi Miwa Toshitaka Nagao Masato Odawara |
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| Affiliation: | 1.Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan; 2.Department of Diabetes, Metabolism, Endocrinology, Tokyo Medical University Hachioji Medical Center, Japan; 3.Department of Anatomic Pathology, Tokyo Medical University, Japan |
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| Abstract: | A 40-year-old woman who had a history of recurrent olfactory neuroblastoma presented with full moon face, central obesity, buffalo hump, impaired glucose tolerance and bilateral cervical lymph node swelling. Laboratory tests showed morbidly elevated levels of adrenocorticotropic hormone (ACTH) and cortisol, which were not suppressed by high-dose (8 mg) dexamethasone. Biopsies of the enlarged cervical lymph nodes revealed ACTH-positive metastatic olfactory neuroblastoma, and ectopic ACTH syndrome was diagnosed. Metyrapone was used to suppress cortisol production and resulted in decreased levels of ACTH and cortisol. Bilateral cervical tumor resection further reduced the ACTH and cortisol levels, accompanied by a reduction in the metyrapone dosage. Cushing''s syndrome was alleviated through ACTH-producing tumor removal. |
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| Keywords: | ectopic ACTH syndrome olfactory neuroblastoma adrenocorticotropic hormone Cushing''s syndrome metyrapone |
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