Ectopic Adrenocorticotropic Hormone Syndrome due to Pancreatic Neuroendocrine Carcinoma

Intern Med. 2025 Jan 15;64(2):237-242. doi: 10.2169/internalmedicine.3672-24. Epub 2024 May 30.

Abstract

A 55-year-old woman presented to her primary care physician with facial and lower leg edema. After being referred to our hospital because of hypothyroidism and hypokalemia on blood tests, she also had elevated adrenocorticotropic hormone (ACTH) and cortisol levels, but a dexamethasone suppression test showed no cortisol suppression. Ectopic ACTH syndrome due to pancreatic neuroendocrine carcinoma (PNEC) was suspected. endoscopic ultrasound-guided fine-needle aspiration was performed, and a histopathological examination of the obtained specimen revealed multiple liver metastases of the PNEC. Imaging after etoposide and cisplatin therapy showed cystic changes in the primary lesions and shrinkage of the liver metastases, and the ACTH levels were within the normal range.

Keywords: ectopic adrenocorticotropic hormone syndrome; endoscopic ultrasound-guided fine-needle aspiration; pancreatic neuroendocrine carcinoma; pancreatic neuroendocrine neoplasms.

Publication types

  • Case Reports

MeSH terms

  • ACTH Syndrome, Ectopic* / diagnosis
  • ACTH Syndrome, Ectopic* / etiology
  • Adrenocorticotropic Hormone / blood
  • Carcinoma, Neuroendocrine* / complications
  • Carcinoma, Neuroendocrine* / diagnosis
  • Carcinoma, Neuroendocrine* / secondary
  • Female
  • Humans
  • Liver Neoplasms / diagnosis
  • Liver Neoplasms / secondary
  • Middle Aged
  • Pancreatic Neoplasms* / complications
  • Pancreatic Neoplasms* / diagnosis

Substances

  • Adrenocorticotropic Hormone