title: The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature.
abstract: OBJECTIVE
  To review the outcome and quality of life at 5 years and more of 37 children treated with radiation therapy combined or not with surgical resection for a craniopharyngioma in a single institution.
  
  
  METHODS
  From January 1969 through December 1992, 37 children received external therapy at the Institut Gustave Roussy (Villejuif, France). Age ranged between 1 and 15 years (mean 7.4), M/F sex ratio was 0.76. In approximately one-half of the cases (18/37), radiation therapy was applied in conjunction with a surgical resection, and in almost one-half of the cases (18/37) as part of a salvage program following local failure. Total dose ranged between 45 and 56 Gy (median 50) given with a conventional fractionation in most children. Survival (S), event-free survival (EFS) were computerized according to the Kaplan-Meier method and prognostic factors for local failure and functional status analyzed. Functional outcome was evaluated according to the Wen score in 4 grades (gr 1: normal with/without hormonal replacement, gr 4: totally dependent, gr 2 and 3: intermediate disabilities).
  
  
  RESULTS
  At the time of analysis, 24 children (65%) were alive with NED, 4 (11%) alive after failure, and 9 (24%) dead of various causes. Following therapy, S and EFS regularly degraded and didn't seem to reach a plateau before 9 years (5 and 10 year S and EFS, respectively, 91, 65, and 78 and 56.5%). This was due to the occurrence of late failures (5 and 8.5 years) and late lethal complications (1 in-field glioblastoma multiforme at 9 years). A significant gain on EFS followed the introduction of modern imaging (p = 0.03), the association of surgical resection with RT (p = 0.01) and of higher doses of radiation superior or equal to 55 Gy (p = 0.05); a similar gain on S was observed in patients with a good initial performance status (p = 0.05). It is remarkable that surgical salvage of local failures following RT could induce prolonged remission in 4 children. Functional outcome was impaired in all but 5 children out of 35 fully evaluable (86%) and related with the initial symptomatology and/or therapy. Endocrinological, visual, neurological functions were affected in 97, 34, and 40%, respectively. It appeared correlated with the initial performance status (p = 0.02) and possibly with a younger age at treatment (p = 0.07).
  
  
  CONCLUSIONS
  Long-term follow-up beyond 5 years is warranted in craniopharyngioma to assess tumor control and functional outcome after radiation therapy. Although this therapeutical modality provides a high cure rate alone or in combination with surgery and even though at the time of failure, further strategies should aim to limit the severe toxicity (i.e., Wen gr 3 + 4) that was observed in more than one-third of our patients.
authors.affiliation: Department of Radiation Oncology, Institut Gustave Roussy, Villejuif, France.
authors.forename: J L
authors.initials: JL
authors.lastname: Habrand
authors.forename: O
authors.initials: O
authors.lastname: Ganry
authors.forename: D
authors.initials: D
authors.lastname: Couanet
authors.forename: V
authors.initials: V
authors.lastname: Rouxel
authors.forename: C
authors.initials: C
authors.lastname: Levy-Piedbois
authors.forename: A
authors.initials: A
authors.lastname: Pierre-Kahn
authors.forename: C
authors.initials: C
authors.lastname: Kalifa
mesh_terms: D002648:Child; D002675:Child, Preschool; D003397:Craniopharyngioma / Q000175:diagnosis / Q000401:mortality / Q000532:radiotherapy*; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D020249:Hormone Replacement Therapy; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D011788:Quality of Life; D016879:Salvage Therapy; D014786:Vision Disorders / Q000209:etiology