Ectopic Adrenocorticotropic Hormone-Producing Metastatic Gastrinoma: A Case Report.

Autor: Tamhane S; Endocrinology, Diabetes and Metabolism, Baptist Health, North Little Rock, USA., Menon LP; Endocrinology, Diabetes and Metabolism, University of Arkansas for Medical Sciences, Little Rock, USA., Edem D; Endocrinology, Diabetes and Metabolism, University of Arkansas for Medical Sciences, Little Rock, USA., Sharma P; Endocrinology, Parkview Medical Center, Pueblo, USA., Maradana J; Endocrinology, Diabetes and Metabolism, Mass General Brigham Wentworth-Douglas Hospital, Dover, USA.
Jazyk: angličtina
Zdroj: Cureus [Cureus] 2023 Sep 15; Vol. 15 (9), pp. e45329. Date of Electronic Publication: 2023 Sep 15 (Print Publication: 2023).
DOI: 10.7759/cureus.45329
Abstrakt: Pancreatic neuroendocrine tumors secreting gastrin and adrenocorticotropic hormone (ACTH) are rare. The presentation of the cases can be varied, making the diagnosis challenging and often delayed. Here, we present a patient who presented with severe hypokalemia and was found to have ectopic Cushing's syndrome. An abdominal CT scan showed a pancreatic lesion with metastatic liver disease. A biopsy of the liver lesion confirmed a metastatic neuroendocrine tumor. The final diagnosis was ectopic ACTH-producing metastatic gastrinoma. Twenty-four-hour urinary cortisol was significantly elevated at 9,790 mcg/24 hours. The excess hormonal secretion was successfully treated with ketoconazole and somatostatin analogs. She was further started on chemotherapy with capecitabine plus temozolomide, which has become the preferred chemotherapy treatment after the results of the recently completed trial. She also received Y90 therapy for metastatic liver disease. The prognosis of metastatic pancreatic neuroendocrine tumors is poor. Multidisciplinary combined therapies can help control disease and improve prognosis. We present an 18-month-long patient follow-up and a literature review of ectopic ACTH-producing metastatic gastrinomas.
Competing Interests: The authors have declared that no competing interests exist.
(Copyright © 2023, Tamhane et al.)
Databáze: MEDLINE