Ectopic ACTH-Dependent Cushing’s Syndrome Emerging at a Late Stage of a Mixed Histology Neuroendocrine Neoplasm: A Case Report

Abstract Introduction: Neuroendocrine neoplasms encompass well-differentiated tumors (NETs) and poorly differentiated carcinomas (neuroendocrine carcinomas [NECs]), which are distinguished by their clinical behavior and molecular characteristics. They can cause paraneoplastic syndromes, such as ecto...

Full description

Saved in:
Bibliographic Details
Published in:Case reports in oncology 2024-10, Vol.17 (1), p.1146-1156
Main Authors: Meyers, Michel, Awada, Ahmad, Karfis, Ioannis, t'Kint de Roodenbeke, Daphné, Couvert, Hugo, Hanssens, Charlotte, Hendlisz, Alain, Driessens, Natacha
Format: Article
Language:English
Subjects:
68ga-dotatate positron emission tomography/computed tomography
Abdomen
Biopsy
Cancer
Case Report
Case reports
Chemotherapy
Creatinine
Cushing syndrome
Dyspnea
ectopic adrenocorticotropic hormone secretion
Expected values
Hormones
Keratin
Laboratories
Lymphatic system
Medical history
Medical imaging
Medical prognosis
Metastasis
mixed neuroendocrine non-neuroendocrine neoplasm
neuroendocrine neoplasm
Neuroendocrine tumors
Patients
peptide receptor radionuclide therapy
Potassium
Pulmonary embolisms
somatostatin receptor
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Introduction: Neuroendocrine neoplasms encompass well-differentiated tumors (NETs) and poorly differentiated carcinomas (neuroendocrine carcinomas [NECs]), which are distinguished by their clinical behavior and molecular characteristics. They can cause paraneoplastic syndromes, such as ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome (CS), necessitating prompt recognition and management due to severe hypercortisolism. Case Presentation: A 66-year-old patient with a 3-year history of metastatic mixed neuroendocrine-non-neuroendocrine neoplasm with a NEC and adenocarcinoma component originating from the vulva presented to the emergency department with dyspnea and fatigue. Upon clinical examination, we found widespread hyperpigmentation, a moon-face appearance, hirsutism, buffalo hump, and muscle atrophy. Laboratory investigations revealed severe hypokalemia (2.3 mmol/L), elevated serum cortisol (1,726 nmol/L) and ACTH (194 ng/L) levels. Urinary free cortisol measurement was 21-fold the upper limit of the reference range (3,614.0 nmol/24 h), and cortisol concentration did not decrease after 1mg-dexamethasone suppression test (1,812 nmol/L for an expected value
ISSN:1662-6575
1662-6575
DOI:10.1159/000540707