Craniopharyngioma in children

Abstract

Purpose: The treatment regimens at presentation and recurrence, the related morbidity, and survival rates were studied for children with craniopharyngioma who were treated at King Faisal Specialist Hospital & Research Center (KFSH&RC).

Methods & materials: From 1975 to 1996, a total of 56 children younger than 18-years-old at diagnosis underwent treatment with surgery and/or radiotherapy for craniopharyngiomas at KFSH&RC. There was evidence that these patients had advanced disease at diagnosis; 36% of 51 patients whose visual status pretreatment was known were either blind or had major bilateral visual defects prior to treatment, and 36% of the 56 patients had diabetes insipidus. This report concentrates on 44 patients who had first definitive resection at KFSH&RC. Treatment policy was total resection if possible (17 patients), if not, subtotal resection (17 patients) or lesser procedures (10 patients). Five patients received postoperative irradiation after first definitive resection.

Results: Ten-year-survival, and progression-free and event-free survival rates were 65%, 39%, and 29%, respectively. There were 9 postoperative deaths; 7 following the first procedure and 2 following resection for relapse, and 3 early deaths at home prior to relapse, following the first resection. None of the 22 patients who underwent less than total resection without postoperative radiation treatment were progression-free at 5 years, whereas the 5 patients who were irradiated remain progression-free.

Conclusion: A more selective approach to total resection, and the routine use of postoperative irradiation following lesser surgical procedures requires evaluation.