The role of radiation therapy in the management of craniopharyngioma: A 25-year experience and review of the literature

Purpose: To review the outcome and quality of life at 5 years and more of 3 7 children treated with radiation therapy combined or not with surgical res ection for a craniopharyngioma in a single institution, Methods and Materials: From January 1969 through December 1992, 37 children received external therapy at the Institut Gustave Roussy (Villejuif, Franc e). Age ranged between 1 and 15 years (mean 7.4), M/F sex ratio was 0.76. I n approximately one-half of the cases (18/37), radiation therapy was applie d in conjunction with a surgical resection, and in almost one-half of the c ases (18/37) as part of a salvage program following local failure, Total do se ranged between 45 and 56 Gy (median 50) given with a conventional fracti onation in most children, Survival (S), event-free survival (EFS) were comp uterized according to the Kaplan-Meier method and prognostic factors for lo cal failure and functional status analyzed. Functional outcome was evaluate d according to the Wen score in 4 grades (gr 1: normal with/without hormona l replacement, gr 4: totally dependent, gr 2 and 3: intermediate disabiliti es). Results: At the time of analysis, 24 children (65%) were alive with SED, 4 (11%) alive after failure, and 9 (24%) dead of various causes, Following th erapy, S and EFS regularly degraded and didn't seem to reach a plateau befo re 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 la te 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 imp aired in all but 5 children out of 35 fully evaluable (86%) and related wit h the initial symptomatology and/or therapy. Endocrinological, visual, neur ological functions were affected in 97, 34, and 40%, respectively. It appea red 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 craniophary ngioma to assess tumor control and functional outcome after radiation thera py, Although this therapeutical modality provides a high cure rate alone or in combination with surgery and even though at the time of failure, furthe r strategies should aim to limit the severe toxicity (i.e., Wen gr 3 + 4) t hat was observed in more than one-third of our patients, (C) 1999 Elsevier Science Inc.