A Rare Case of Ectopic Adrenocorticotropic Hormone Syndrome with Recurrent Olfactory Neuroblastoma

Hironori Abe; Hirotsugu Suwanai; Nodoka Kambara; Koji Sano; Junpei Shikuma; Hiroaki Akaoka; Akira Kanazawa; Hideaki Hirai; Takashi Miwa; Toshitaka Nagao; Masato Odawara

doi:10.2169/internalmedicine.2897-19

This article has now been updated. Please use the final version.

A Rare Case of Ectopic Adrenocorticotropic Hormone Syndrome with Recurrent Olfactory Neuroblastoma

Hironori Abe, Hirotsugu Suwanai, Nodoka Kambara, Koji Sano, Junpei Shikuma, Hiroaki Akaoka, Akira Kanazawa, Hideaki Hirai, Takashi Miwa, Toshitaka Nagao, Masato Odawara

Author information

Hironori Abe
Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan
Hirotsugu Suwanai
Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan
Nodoka Kambara
Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan
Koji Sano
Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan
Junpei Shikuma
Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan
Hiroaki Akaoka
Department of Diabetes, Metabolism, Endocrinology, Tokyo Medical University Hachioji Medical Center, Japan
Akira Kanazawa
Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan
Hideaki Hirai
Department of Anatomic Pathology, Tokyo Medical University, Japan
Takashi Miwa
Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan
Toshitaka Nagao
Department of Anatomic Pathology, Tokyo Medical University, Japan
Masato Odawara
Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Japan

Keywords: Ectopic ACTH syndrome, olfactory neuroblastoma, adrenocorticotropic hormone, Cushing's syndrome, metyrapone

JOURNAL OPEN ACCESS Advance online publication

Article ID: 2897-19

DOI https://doi.org/10.2169/internalmedicine.2897-19

The final version of this article is now available: Vol. 60 (2021), No. 1 pp. 105-109

Details

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 ACTH and cortisol, which were not suppressed by high-dose (8 mg) dexamethasone. Biopsies of the enlarged cervical lymph nodes revealed adrenocorticotropic hormone (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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