ACTH-secreting pancreatic neuroendocrine neoplasms: A case-series.
ACTH
Cushing's syndrome
ectopic Cushing's
neuroendocrine tumor
pancreatic neuroendocrine neoplasms
Journal
Journal of neuroendocrinology
ISSN: 1365-2826
Titre abrégé: J Neuroendocrinol
Pays: United States
ID NLM: 8913461
Informations de publication
Date de publication:
Oct 2023
Oct 2023
Historique:
revised:
29
07
2023
received:
07
03
2023
accepted:
15
08
2023
pubmed:
9
9
2023
medline:
9
9
2023
entrez:
9
9
2023
Statut:
ppublish
Résumé
Ectopic Cushing's syndrome (CS) occurs rarely in patients with pancreatic neuroendocrine neoplasms. Early recognition of symptoms is critical given the high morbidity and mortality associated with CS. A database of pancreatic neuroendocrine neoplasms (NENs) seen at the Moffitt Cancer Center between 1/2008 and 4/2022 was reviewed and cases of ectopic CS were identified. Information was extracted on tumor characteristics, clinical signs and symptoms, therapies, and outcomes. Thirteen cases were identified, ranging in age from 16 to 65 years at the initial time of diagnosis (median 42). Twelve of 13 patients had metastatic tumors at presentation. All were well-differentiated at diagnosis although two were described as transformed to poorly differentiated on rebiopsy. A total of 4 patients also experienced Zollinger-Ellison syndrome. Three patients underwent bilateral adrenalectomy to manage uncontrolled CS. Median overall survival of was 56 months from the time of initial cancer diagnosis but only 18 months from diagnosis of CS. Our study showed that ectopic CS is a highly morbid condition when occurring in pancreatic NENs and is associated with aggressive disease. Bilateral adrenalectomy can be considered for syndrome control. To our knowledge, this is the largest institutional case-series of ACTH-secreting metastatic pancreatic NEN.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e13336Informations de copyright
© 2023 British Society for Neuroendocrinology.
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