title: Hypothalamic-pituitary Langerhans cell histiocytosis: a diagnostic challenge.
abstract: Four cases of hypothalamic-pituitary Langerhans cell histiocytosis (LCH) are reported, highlighting the expanding spectrum of clinical and magnetic resonance imaging (MRI) features in adults. The diagnostic challenge of hypothalamic-pituitary LCH is emphasized in cases revealed as supra-sellar tumors with panhypopituitarism or as isolated central diabetes insipidus. Diagnosis is confirmed by histological examination showing infiltration with CD1a positive histiocytes. General guidelines for diagnosis procedure are drawn out, including the neurosurgical biopsy in particular cases.
authors.affiliation: Department of Endocrinology, Hôpital de l'Archet 1, BP 3079, 06202 Nice Cedex 3 France. hieronimus.s@chu-nice.fr
authors.forename: S
authors.initials: S
authors.lastname: Hiéronimus
authors.forename: Y
authors.initials: Y
authors.lastname: Hadjali
authors.forename: A
authors.initials: A
authors.lastname: Fredenrich
authors.forename: P
authors.initials: P
authors.lastname: Paquis
authors.forename: S
authors.initials: S
authors.lastname: Chanalet
authors.forename: A
authors.initials: A
authors.lastname: Grimaud
authors.forename: J
authors.initials: J
authors.lastname: Michiels
authors.forename: P
authors.initials: P
authors.lastname: Fenichel
mesh_terms: D000328:Adult; D000368:Aged; D001766:Blindness / Q000209:etiology; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D010900:Pituitary Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D010911:Pituitary Neoplasms / Q000175:diagnosis; D012076:Renal Agents / Q000627:therapeutic use
chemical_list: D012076:Renal Agents; D003894:Deamino Arginine Vasopressin