title: Fulminant ectopic Cushing syndrome in a patient with metastatic neuroendocrine carcinoma and Crohn's disease.
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.
authors.affiliation: Institute of Endocrinology, Beilinson Hospital, Rabin Medical Center, Petah Tikva, Israel.
authors.forename: Alexander
authors.initials: A
authors.lastname: Gorshtein
authors.forename: Yulia
authors.initials: Y
authors.lastname: Strenov
authors.forename: Eytan
authors.initials: E
authors.lastname: Mor
authors.forename: Ilan
authors.initials: I
authors.lastname: Shimon
authors.forename: Simona
authors.initials: S
authors.lastname: Grozinsky-Glasberg
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000209:etiology*; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D018278:Carcinoma, Neuroendocrine / Q000150:complications / Q000378:metabolism / Q000473:pathology; D003424:Crohn Disease; D017809:Fatal Outcome; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000150:complications / Q000378:metabolism / Q000473:pathology; D006801:Humans; D009362:Neoplasm Metastasis / Q000473:pathology*
chemical_list: D000324:Adrenocorticotropic Hormone