Maximal removal of pediatric suprasellar craniopharyngioma by extended transsphenoidal approach - Reconstituted hypothalamic function and long-term tumor control

Object: Long-term tumor control and maintenance of good quality of life are sometimes difficult to achieve in the treatment of craniopharyngioma despite modern microsurgery techniques. The transsphenoidal approach for tumors with large suprasellar extension has not been fully established for pediatric patients, because of their narrow nostrils and undeveloped sphenoidal sinus. Methods: Seventeen consecutive pediatric patients, 6 boys and 11 girls aged from 3 to 19 years (mean 12.0 years), with craniopharyngioma and extension to suprasellar cistern were treated through the extended transsphenoidal approach. Surgery was intended to achieve maximum removal without causing neurological deficits. And the closest attention was paid to preserve the lateral walls of the third ventricle. Removal rate, pituitary function, and adverse events were evaluated. Results: Gross total removal was achieved in 14 of 17 patients, and all patients were discharged without autonomic and/or thermal disturbances except for 2 patients with preoperative hypothalamic disturbance. All patients with gross total removal have remained disease-free in the follow-up period (mean 38.1 months). Anti-diuretic hormone administration was discontinued in 8 of the 17 patients in the follow-up period, although glucocorticoid administration could not be discontinued in any patient except for one patient without involvement of the pituitary stalk. No patients suffered new hypothalamic disturbance, and all patients could return to school life or obtain employment. Conclusion: High rate of gross total removal with the compatibility of good quality of life was achieved for pediatric craniopharyngioma patients through the extended transsphenoidal approach. Long-term risk analysis is required in the future.