Fulminant ectopic Cushing syndrome in a patient with metastatic neuroendocrine carcinoma and Crohn's disease

Alexander Gorshtein, Yulia Strenov, Eytan Mor, Ilan Shimon, Simona Grozinsky-Glasberg*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) have been rarely reported in patients with Crohn's disease, being usually small and incidentally detected in areas uninvolved by the inflammatory process. We describe the case of a young female patient with Crohn's disease and a fulminant Cushing's syndrome induced by the ectopic secretion of adrenocorticotropic hormone (ACTH) by an aggressive gastrointestinal neuroendocrine carcinoma (NEC). Despite a multi-therapeutic approach, including the administration of multiple courses of chemotherapy, hypo-cortisolemic agents, somatostatin analogues, as well as the performance of bilateral adrenal vein embolization followed by bilateral adrenalectomy, patient's condition progressively deteriorated and she died nine months after the diagnosis of NEC due to liver failure. The available literature addressing the possible connection between Crohn's disease and NEC is discussed in detail.

Original languageEnglish
Pages (from-to)735-740
Number of pages6
JournalNeuroendocrinology Letters
Volume32
Issue number6
StatePublished - 2011

Keywords

  • Carcinoid
  • Cortisol
  • Crohn's disease
  • Ectopic Cushing's syndrome
  • Immunosuppression
  • Neuroendocrine tumor

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