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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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.[1]


  1. Clinical outcome in children with craniopharyngioma treated with primary surgery and radiotherapy deferred until relapse. Kalapurakal, J.A., Goldman, S., Hsieh, Y.C., Tomita, T., Marymont, M.H. Med. Pediatr. Oncol. (2003) [Pubmed]
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