title: Long-term outcome of hypothalamic pituitary tumors in Langerhans cell histiocytosis.
abstract: BACKGROUND
  Hypothalamic-pituitary (HP) disease is the most common CNS manifestation of Langerhans cell histiocytosis (LCH) frequently leading to diabetes insipidus (DI) and anterior pituitary hormone deficiencies (APD). On MRI, loss of the normal posterior pituitary signal and thickening of the pituitary stalk have been described, as well as neurodegenerative signal changes associated with neuropsychological disabilities in some patients. The influence of therapy on the long-term course of HP tumors and neurodegeneration (ND) is not well-understood.
  
  
  METHODS
  In this retrospective survey we focused on patients with LCH and HP disease with clinical and MRI data available at diagnosis of HP disease and at least three follow up investigations. We collected clinical and MRI follow-up information for central review and analysis.
  
  
  RESULTS
  We identified 22 patients with HP tumors (HPT) registered at the LCH study center. Many different treatment regimens were applied for variable periods, with more than one regimen in most patients. Regression of the tumor was seen in the majority, but all patients had APD or ND on MRI at last follow up. In none of the patients APD and ND regressed or resolved. A deterioration of radiological ND was noted in 17 patients leading to overt clinical neuropsychological impairment in five.
  
  
  CONCLUSIONS
  Patients with HPT appear to be at high risk to develop permanent neuroendocrine consequences. Coordinated studies for patients with LCH and HP disease including thorough MRI monitoring and neuropsychological tests are needed.
authors.affiliation: St. Anna Children's Cancer Research Institute, Vienna, Austria. bernhard.fahrner@ccri.at
authors.forename: Bernhard
authors.initials: B
authors.lastname: Fahrner
authors.forename: Helmut
authors.initials: H
authors.lastname: Prosch
authors.forename: Milen
authors.initials: M
authors.lastname: Minkov
authors.forename: Martha
authors.initials: M
authors.lastname: Krischmann
authors.forename: Helmut
authors.initials: H
authors.lastname: Gadner
authors.forename: Daniela
authors.initials: D
authors.lastname: Prayer
authors.forename: Nicole
authors.initials: N
authors.lastname: Grois
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003919:Diabetes Insipidus / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D010908:Pituitary Hormones, Anterior / Q000172:deficiency*; D010911:Pituitary Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy; D011859:Radiography; D012189:Retrospective Studies; D013997:Time Factors
chemical_list: D010908:Pituitary Hormones, Anterior