CRANIOPHARYNGIOMA IN CHILDREN

Purpose: The treatment regimens at presentation and recurrence, the re lated morbidity, and survival rates were studied for children with cra niopharyngioma who were treated at King Faisal Specialist Hospital & R esearch Center (KFSH&RC). Methods & Materials: From 1975 to 1996, a to tal of 56 children younger than 18-years-old at diagnosis underwent tr eatment with surgery and/or radiotherapy for craniopharyngiomas at KFS H&RC. There was evidence that these patients had advanced disease at d iagnosis; 36% of 51 patients whose visual status pretreatment was know n were either blind or had major bilateral visual defects prior to tre atment, and 36% of the 56 patients had diabetes insipidus. This report concentrates on 44 patients who had first definitive resection at KFS H&RC. Treatment policy was total resection if possible (17 patients), if not, subtotal resection (17 patients) or lesser procedures (10 pati ents). Five patients received postoperative irradiation after first de finitive resection. Results: Ten year-survival, and progression-free a nd event-free survival rates were 65%, 39%, and 29%, respectively. The re were 9 postoperative deaths; 7 following the first procedure and 2 following resection for relapse, and 3 early deaths at home prior to r elapse, following the first resection. None of the 22 patients who und erwent less than total resection without postoperative radiation treat ment were progression-free at 5 years, whereas the 5 patients who were irradiated remain progression-free. Conclusion: A more selective appr oach to total resection, and the routine use of postoperative irradiat ion following lesser surgical procedures requires evaluation. (C) 1998 Elsevier Science Inc.