title: Childhood craniopharyngioma. Recent advances in diagnosis, treatment and follow-up.
abstract: BACKGROUND
  Craniopharyngioma are embryogenic malformations of the sellar area. With an overall incidence of 0.5-2 new cases per million population per year, 30-50% of all cases occur in childhood. Overall survival rates are high. However, quality of life (QoL) is substantially reduced in many survivors due to sequelae such as extreme obesity caused by hypothalamic lesions.
  
  
  METHODS
  Based on retrospective analysis of 306 patients with childhood craniopharyngioma (HIT ENDO), we found that QoL was negatively related to hypothalamic involvement, tumor size and the number of neurosurgical interventions.
  
  
  RESULTS
  Irradiation had no significant impact on long-term QoL. The prospective surveillance of 98 patients in KRANIOPHARYNGEOM 2000 revealed frequent and early events in terms of tumor relapse after apparently complete resection (EFS: 0.60 +/- 0.09 at 3 years) and tumor progression after incomplete resection (EFS: 0.22 +/- 0.09).
  
  
  CONCLUSIONS
  We conclude that radical surgery in patients with hypothalamic involvement has a major negative impact on long-term QoL. Innovative treatment strategies are warranted to improve QoL in these patients at risk. Accordingly, the appropriate time point of irradiation after incomplete resection will be analyzed in KRANIOPHARYNGEOM 2007.
authors.affiliation: Department of Pediatrics, Pediatric Hematology and Oncology, Klinikum Oldenburg GmbH, Oldenburg, Germany. mueller.hermann@klinikum-oldenburg.de
authors.forename: Hermann L
authors.initials: HL
authors.lastname: Muller
mesh_terms: D002648:Child; D003397:Craniopharyngioma / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D005500:Follow-Up Studies; D006801:Humans; D007031:Hypothalamus / Q000503:physiopathology; D009765:Obesity / Q000209:etiology / Q000503:physiopathology; D010911:Pituitary Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D011379:Prognosis; D011788:Quality of Life; D012189:Retrospective Studies