title: Xanthogranuloma, Rathke's cyst, and childhood craniopharyngioma: results of prospective multinational studies of children and adolescents with rare sellar malformations.
abstract: BACKGROUND
  Craniopharyngioma (CP), Rathke's cyst (RC), and xanthogranuloma (XG) are closely related rare sellar masses. Treatment strategies in children lack consensus.
  
  
  OBJECTIVE
  Our objective was to study clinical manifestations and treatment-related outcome in RC, XG, and CP patients.
  
  
  METHODS
  We conducted a multicenter surveillance trial.
  
  
  METHODS
  Inclusion criteria were 1) histological diagnosis of CP, XG, or RC and 2) diagnosis at age of 18 yr or less. A total of 117 CP, 14 XG (2001-2006), and 14 RC (1996-2006) were recruited.
  
  
  RESULTS
  Overall survival (OS), event-free survival (EFS), and quality of life (QoL) were evaluated.
  
  
  RESULTS
  The 5-yr OS rates were 1.00 ± 0.00 in RC and XG and 0.97 ± 0.02 in CP. The 5-yr EFS rates were 0.85 ± 0.10 in RC, 1.00 ± 0.00 in XG, and 0.50 ± 0.05 in CP. Surgical resection of XG results in complete remission without recurrence. Recurrences occur in RC (14%) and CR (59%) but can be efficiently treated by irradiation, reoperation, and/or intracystic treatment. Severe hypothalamic sequelae such as obesity and others affecting QoL are predominant in CP due to presurgical involvement (59%) and postsurgical lesions (44%) of posterior hypothalamic structures. Centers with lower neurosurgery patient load use more radical surgical approaches to treat CP, resulting in higher rates of obesity and reduced QoL. Despite 46% anterior hypothalamic involvement, severe obesity is not encountered in XG.
  
  
  CONCLUSIONS
  Treatment of choice in XG and RC is radical surgery. In CP involving hypothalamic structures, less radical surgical approaches preserving hypothalamic integrity are recommended. Due to frequent relapses, regular imaging during follow-up is recommended for CP and RC. Treatment of patients with sellar masses should be confined to experienced multidisciplinary teams.
authors.affiliation: Department of Pediatrics, Klinikum Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, Germany. mueller.hermann@klinikum-oldenburg.de
authors.forename: Hermann L
authors.initials: HL
authors.lastname: Müller
authors.forename: Ursel
authors.initials: U
authors.lastname: Gebhardt
authors.forename: Andreas
authors.initials: A
authors.lastname: Faldum
authors.forename: Monika
authors.initials: M
authors.lastname: Warmuth-Metz
authors.forename: Torsten
authors.initials: T
authors.lastname: Pietsch
authors.forename: Fabian
authors.initials: F
authors.lastname: Pohl
authors.forename: Gabriele
authors.initials: G
authors.lastname: Calaminus
authors.forename: Niels
authors.initials: N
authors.lastname: Sörensen
mesh_terms: D000293:Adolescent; D020863:Central Nervous System Cysts / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003397:Craniopharyngioma / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D062665:Epidemiological Monitoring; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010911:Pituitary Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D011446:Prospective Studies; D011788:Quality of Life; D012658:Sella Turcica / Q000473:pathology / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome; D014972:Xanthogranuloma, Juvenile / Q000401:mortality* / Q000473:pathology / Q000628:therapy