title: Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion from a Meckel diverticulum neuroendocrine tumor: case report.
abstract: BACKGROUND
  Ectopic production of adrenocorticotropic hormone (ACTH) by neuroendocrine tumours (NET) is a rare condition, occult presentations often hampering the diagnosis. Although NET are relatively frequent in the ileon and Meckel diverticulum, we describe the first Cushing's syndrome due to ectopic adrenocorticotropic syndrome (CS-EAS) arising from a Meckel diverticulum.
  
  
  METHODS
  A 44-year-old man was admitted with recent onset of diabetes, myopathy, edema and hypokalemic metabolic alkalosis consistent with Cushing's syndrome. Both basal and dynamic laboratory evaluation suggested CS-EAS. Laboratory testing also showed high serum levels of chromogranin A (CgA) and urinary 5-hydroxyindoleacetic acid (5HIAA). Pituitary and neck/thorax/abdomen/pelvis imaging proved to be normal, while somatostatin analogue ((99m)Tc-HYNIC-TOC) scintigraphy revealed increased focalized ileum uptake on the right iliac fossa. Pre-operative ketoconazole and sandostatin treatment controlled the hypercortisolism within a month. Pathological analysis of the resected submucosal 1.8 cm tumour of the Meckel diverticulum and a metastatic local lymph node confirmed a well differentiated neuroendocrine tumour (grade I), whereas immunohistochemistry was positive for ACTH, chromogranin A and synaptophysin. Post-operative clinical and biochemical resolution of Cushing's syndrome was followed by normalization of both CgA and 5HIAA, which were maintained at the 6 month follow-up.
  
  
  CONCLUSIONS
  The identification, characterization and follow-up of this rare cause of ectopic ACTH secretion is important in order to assess the long-term prognostic and management.
authors.affiliation: "C.I. Parhon" National Institute of Endocrinology, 34-36, Aviatorilor Boulevard, sector 1, 011863, Bucharest, Romania.|"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
authors.forename: Diana Loreta
authors.initials: DL
authors.lastname: Paun
authors.affiliation: "C.I. Parhon" National Institute of Endocrinology, 34-36, Aviatorilor Boulevard, sector 1, 011863, Bucharest, Romania. vija_lm@yahoo.com.
authors.forename: Lavinia
authors.initials: L
authors.lastname: Vija
authors.affiliation: Braila Department Hospital, Braila, Bucharest, Romania.
authors.forename: Emilia
authors.initials: E
authors.lastname: Stan
authors.affiliation: "C.I. Parhon" National Institute of Endocrinology, 34-36, Aviatorilor Boulevard, sector 1, 011863, Bucharest, Romania.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Banica
authors.affiliation: "C.I. Parhon" National Institute of Endocrinology, 34-36, Aviatorilor Boulevard, sector 1, 011863, Bucharest, Romania.
authors.forename: Elena
authors.initials: E
authors.lastname: Bobeica
authors.affiliation: "C.I. Parhon" National Institute of Endocrinology, 34-36, Aviatorilor Boulevard, sector 1, 011863, Bucharest, Romania.
authors.forename: Dana
authors.initials: D
authors.lastname: Terzea
authors.affiliation: "C.I. Parhon" National Institute of Endocrinology, 34-36, Aviatorilor Boulevard, sector 1, 011863, Bucharest, Romania.|"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
authors.forename: Catalina
authors.initials: C
authors.lastname: Poiana
authors.affiliation: "C.I. Parhon" National Institute of Endocrinology, 34-36, Aviatorilor Boulevard, sector 1, 011863, Bucharest, Romania.|"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
authors.forename: Corin
authors.initials: C
authors.lastname: Badiu
authors.affiliation: "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.|General Surgery Department, Bucharest Emergency Hospital, Bucharest, Romania.
authors.forename: Sorin
authors.initials: S
authors.lastname: Paun
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000175:diagnosis* / Q000473:pathology; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D003346:Corticotropin-Releasing Hormone / Q000378:metabolism; D003480:Cushing Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D003924:Diabetes Mellitus, Type 2 / Q000097:blood / Q000209:etiology / Q000473:pathology*; D018450:Disease Progression; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use; D007654:Ketoconazole / Q000627:therapeutic use; D008297:Male; D008467:Meckel Diverticulum / Q000175:diagnosis* / Q000473:pathology; D008687:Metformin / Q000627:therapeutic use; D009135:Muscular Diseases / Q000097:blood / Q000209:etiology / Q000473:pathology*; D018358:Neuroendocrine Tumors / Q000175:diagnosis* / Q000473:pathology; D011877:Radionuclide Imaging / Q000379:methods; D013004:Somatostatin / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D007004:Hypoglycemic Agents; D013004:Somatostatin; D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone; D008687:Metformin; D007654:Ketoconazole