title: Cushing's syndrome associated with a nested stromal epithelial tumor of the liver: hormonal, immunohistochemical, and molecular studies.
abstract: BACKGROUND
  Ectopic ACTH syndrome (EAS) is principally associated with aggressive malignant tumors but also with neuroendocrine tumors of good prognosis. Recently, rare nonhepatocytic nested stromal and epithelial tumors (NSET) were characterized by their possible association with Cushing's syndrome of which biochemical and physiopathological features were still incompletely studied.
  
  
  OBJECTIVE
  To describe the clinical and hormonal characteristics of an EAS originating from a liver NSET and further understand the mechanism of cortisol overproduction.
  
  
  METHODS
  This is a clinical case report from the Endocrinology Department of Caen University Hospital, France.
  
  
  METHODS
  A 17-year-old female patient was found to have a large liver NSET with mild Cushingoid clinical features and intense biological hypercortisolism but moderate ACTH secretion. Resection of the tumor was curative with a 30-month follow-up.
  
  
  RESULTS
  The epithelial component of the tumor coexpressed ACTH mildly, corticotropin-releasing hormone (CRH) strongly, and 11beta-hydroxysteroid dehydrogenase at a level comparable with normal human hepatocytes.
  
  
  CONCLUSIONS
  Liver NSET is a new cause of EAS, which may evoke hypercortisolism by multiple biochemical pathways.
authors.affiliation: Unité Fonctionnelle d'Endocrinologie et Maladies Métaboliques, Department of Medicine, CHU Côte de Nacre, 14033 Caen Cedex, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Rod
authors.forename: Manuela
authors.initials: M
authors.lastname: Voicu
authors.forename: Laurence
authors.initials: L
authors.lastname: Chiche
authors.forename: Céline
authors.initials: C
authors.lastname: Bazille
authors.forename: Hervé
authors.initials: H
authors.lastname: Mittre
authors.forename: Estelle
authors.initials: E
authors.lastname: Louiset
authors.forename: Yves
authors.initials: Y
authors.lastname: Reznik
mesh_terms: D042842:11-beta-Hydroxysteroid Dehydrogenases / Q000096:biosynthesis / Q000235:genetics; D000182:ACTH Syndrome, Ectopic / Q000150:complications* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000378:metabolism / Q000652:urine; D003346:Corticotropin-Releasing Hormone / Q000096:biosynthesis / Q000235:genetics; D003480:Cushing Syndrome / Q000209:etiology* / Q000235:genetics / Q000378:metabolism; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism / Q000652:urine; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000150:complications* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D011333:Pro-Opiomelanocortin / Q000096:biosynthesis / Q000235:genetics; D012334:RNA, Neoplasm / Q000096:biosynthesis / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: D012334:RNA, Neoplasm; D011333:Pro-Opiomelanocortin; D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone; D042842:11-beta-Hydroxysteroid Dehydrogenases; D006854:Hydrocortisone