title: Clinical outcome in children with craniopharyngioma treated with primary surgery and radiotherapy deferred until relapse.
abstract: BACKGROUND
  To report the clinical outcome in children with craniopharyngioma following primary surgery and deferred radiotherapy at relapse.
  
  
  METHODS
  Twenty-five children with craniopharyngioma were treated with primary surgery. Total resection was achieved in 19 children (76%), while in 24% total resection was not achieved due to tumor adhesion to adjacent critical structures. None of these children received radiation therapy immediately after total or sub-total resection. Radiotherapy was delivered at the time of relapse in 11 patients (44%).
  
  
  RESULTS
  The median follow-up from primary surgery was 10 years (3-16 years). The 14 year overall survival was 100%. Tumor recurrence was observed in (12/25) 48% at a median interval of 17 months. Tumor recurrence following total resection was 6/19 (32%) compared to 100% (6/6) following sub-total resection, and radiotherapy. The 2, 3, and 6 years relapse-free survival following initial surgery was 72, 55, and 50%, respectively. Univariate analysis revealed only extent of surgery to be significant for local recurrence (P < 0.0001). The sequelae observed in these patients included panhypopituitarism (100%), appetite disorders and hypothalamic obesity (32%), neuropsychological and behavioral disorders (20%), and sleep disorders (12%). Majority of children with non-endocrine complications had a local recurrence requiring further surgery and radiotherapy.
  
  
  CONCLUSIONS
  The two standard treatment options in children with craniopharyngioma are primary surgery and sub-total resection followed by radiotherapy. In certain subgroups of patients such as those with large tumors and hypothalamic extension, primary surgery is associated with a high incidence of complications and high failure rates. We recommend utilization of an individualized risk-based treatment approach, that attempts to maximize cure rates without compromising long-term functional outcome in children with craniopharyngiomas.
authors.affiliation: Division of Radiation Oncology, Northwestern Memorial Hospital, Children's Memorial Hospital, Chicago, Illinois 60611, USA.
authors.forename: John A
authors.initials: JA
authors.lastname: Kalapurakal
authors.forename: Stewart
authors.initials: S
authors.lastname: Goldman
authors.forename: Y C
authors.initials: YC
authors.lastname: Hsieh
authors.forename: Tadanori
authors.initials: T
authors.lastname: Tomita
authors.forename: Maryanne H
authors.initials: MH
authors.lastname: Marymont
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003397:Craniopharyngioma / Q000532:radiotherapy* / Q000601:surgery*; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007018:Hypopituitarism / Q000209:etiology; D007027:Hypothalamic Diseases / Q000209:etiology; D007223:Infant; D008297:Male; D009361:Neoplasm Invasiveness; D009765:Obesity / Q000209:etiology; D010911:Pituitary Neoplasms / Q000532:radiotherapy* / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome