title: Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review.
abstract: BACKGROUND
  Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis characterized by xanthomatous tissue infiltration with CD68(+) CD1a(-) foamy histiocytes. Endocrine problems, such as diabetes insipidus and hypogonadotropic hypogonadism, frequently occur in ECD, but bilateral adrenal infiltration has rarely been reported in this disease.
  
  
  OBJECTIVE
  The aim of the study was to describe adrenal and periadrenal infiltration in ECD.
  
  
  METHODS
  A total of 22 patients with ECD undergoing systematic computed tomography to search for signs of adrenal enlargement participated in the study.
  
  
  RESULTS
  Of the 22 patients with ECD, seven (31.8%) displayed adrenal infiltration on computed tomography. In one case, autopsy confirmed that the adrenal enlargement was due to foamy histiocyte infiltration in the adrenal glands. Possible adrenal insufficiency was assessed in five of the seven patients. One developed signs of adrenal insufficiency, which was confirmed by adrenocorticotropin stimulation tests. Adrenal involvement was reported in only 15 of the 240 ECD cases published up to May 2006. This frequency is significantly lower than that in our series (P = 0.0008; Fisher's exact test).
  
  
  CONCLUSIONS
  Physicians should be aware of ECD as a possible cause of morphological changes in adrenal size and infiltration.
authors.affiliation: Service de Médecine Interne, Hôpital Pitié-Salpêtrière, 47-83 Bld de l'Hôpital, Paris, France. julien.haroche@psl.aphp.fr
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Philippe
authors.initials: P
authors.lastname: Touraine
authors.forename: Danielle
authors.initials: D
authors.lastname: Seilhean
authors.forename: Claire
authors.initials: C
authors.lastname: Graef
authors.forename: Béatrice
authors.initials: B
authors.lastname: Birmelé
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.forename: Philippe
authors.initials: P
authors.lastname: Cluzel
authors.forename: Philippe A
authors.initials: PA
authors.lastname: Grenier
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
mesh_terms: D000307:Adrenal Gland Diseases / Q000000981:diagnostic imaging* / Q000473:pathology*; D000311:Adrenal Glands / Q000000981:diagnostic imaging / Q000473:pathology; D000328:Adult; D000368:Aged; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging* / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed