• John A Kalapurakal, Stewart Goldman, Y C Hsieh, Tadanori Tomita, and Maryanne H Marymont.
• Division of Radiation Oncology, Northwestern Memorial Hospital, Children's Memorial Hospital, Chicago, Illinois 60611, USA.
• Med. Pediatr. Oncol. 2003 Apr 1; 40 (4): 214-8.

BackgroundTo report the clinical outcome in children with craniopharyngioma following primary surgery and deferred radiotherapy at relapse.ProcedureTwenty-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%).ResultsThe 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.ConclusionsThe 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.Copyright 2003 Wiley-Liss, Inc.

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