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

Intern Med. 2021 Jan 1;60(1):105-109. doi: 10.2169/internalmedicine.2897-19. Epub 2020 Sep 12.

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.

Keywords: Cushing's syndrome; adrenocorticotropic hormone; ectopic ACTH syndrome; metyrapone; olfactory neuroblastoma.

Publication types

  • Case Reports

MeSH terms

  • ACTH Syndrome, Ectopic* / diagnosis
  • Adrenocorticotropic Hormone
  • Esthesioneuroblastoma, Olfactory* / complications
  • Esthesioneuroblastoma, Olfactory* / diagnosis
  • Humans
  • Hydrocortisone
  • Nasal Cavity
  • Neoplasm Recurrence, Local
  • Nose Neoplasms* / complications
  • Nose Neoplasms* / diagnosis

Substances

  • Adrenocorticotropic Hormone
  • Hydrocortisone