ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2019) 63 EP17 | DOI: 10.1530/endoabs.63.EP17

A rare case of ectopic ACTH syndrome due to recurrence of olfactory neuroblastoma

Hironori Abe1, Hirotsugu Suwanai1, Takuya Ishikawa1, Fumiyoshi Yakou1, Natsuko Hara1, Hideaki Hirai2, Toshitaka Nagao2, Takashi Miwa1 & Masato Odawara1


1Department of Diabetes, Metabolism, Endocrinology, Rheumatology and Collagen Diseases, Tokyo Medical University, Tokyo, Japan; 2Department of Anatomic Pathology, Tokyo Medical University, Tokyo, Japan.


We hereby report a rare case of ectopic adrenocorticotropic hormone (ACTH) syndrome due to recurrence of olfactory neuroblastoma which has few published reports. The female patient had surgery of olfactory neuroblastoma at 31 years old without the symptom of Cushing’s syndrome. Hypercortisolemia during treatment for recurrence of olfactory neuroblastoma were observed at 40 years old. The clinical findings were as followed; full moon face, central obesity, buffalo hump, and impaired glucose tolerance. Tumors of size 40–50 mm considered to be metastases from olfactory neuroblastoma were confirmed by lateral cervical Computed Tomography (CT). ACTH staining was positive in biopsy specimen and that the patient was diagnosed as ectopic ACTH syndrome from metastatic olfactory neuroblastoma. ACTH was not detected in the specimen of craniotomy at 31 years old. Therefore, it was considered that the ACTH producing tumor was manifested at the time of this recurrence at 40 years old. In order to suppress cortisol production, methyrapone started and the blood cortisol level decreased. Furthermore, resection of a bilateral cervix tumor, that is supposed to be an ACTH production site, decreased serum level of ACTH and cortisol level and the dose of methyrapone. Ectopic ACTH producing tumors present featured clinical findings of Cushing’s syndrome. The common origins of tumors are small cell lung tumors, carcinoids and pancreatic neuroendocrine tumors. Few cases of ACTH producing olfactory neuroblastoma were reported in database. Moreover Cushing’s syndrome is basically considered to have a good prognosis, but it can cause various complications such as infection, hypertension, diabetes, dyslipidemia and cardiovascular disease. Therefore, it is important to intervene at an early stage to prevent complications.

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