Clinical outcome in children with craniopharyngioma treated with primary surgery and radiotherapy deferred until relapse

Background. To report the clinical outcome in children with craniopharyngioma following primary surgery and deferred radiotherapy at relapse. Procedure. Twenty-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%). Results. The 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. Conclusions. The 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.