Craniopharyngiomas in children: surgical experience at Children's Memorial Hospital

Abstract

Objectives: Craniopharyngioma during childhood poses difficulty in management because of the high incidence of surgical complications and treatment failure. In order to identify less detrimental and more effective treatment, a personal series of craniopharyngioma was reviewed in regard to various clinical factors, patient factors (age and sex), tumor factors (location and extension, relationship with chiasm, and hydrocephalus), and therapeutic modes [extent of resection and radiation therapy (RT)].

Materials and methods: Fifty-four childhood craniopharyngiomas treated from 1984 to 2003 were reviewed. Preoperative neuroimaging studies were classified depending upon tumor location and extension. In this series of 54 patients, 43 had total tumor resection and 11 had subtotal resection. Of the total resection group, ten showed evidence of residual tumor on postoperative neuroimaging studies. Following the initial resection, 46 did not have RT whereas 8 with subtotal resection received RT.

Results: There were no surgical deaths. Postoperative complications included pseudoaneurysm in 1, hemiparesis in 3, severe obesity in 5, panhypopituitarism in 50, and worsening of visual function in 7. During follow-up ranging from 12 months to 21 years, 24 patients had recurrence. Of the 33 patients with radiographic total resection, 9 (27.3%) had recurrence. Among the patients with total resection but radiographic residual and those with subtotal resection, the craniopharyngioma recurred in 90% and 100%, respectively. Three (37.5%) of eight patients with subtotal resection with RT had recurrence. Overall recurrence-free survival was 62% at 5 years and 49% at 10 years. The sex and age, location and extension of the tumor, nature of the optic chiasm, and hydrocephalus did not influence survival with statistical significance. However, the extent of surgical resection and use of RT showed significant differences for survival. Patients with total resection had a recurrence-free survival rate of 83% and 70% at 5 and 10 years, respectively. Patients with subtotal resection with RT had 71% at 5 years and 36% at 10 years. Patients who had subtotal resection or radiographically residual tumor without RT had a recurrence-free survival rate of only 9%. Among 22 patients whose recurrent tumor was treated with RT, a second recurrence-free survival rate was 90% at 5 years.

Conclusion: Total resection provided the best outcome. However, recurrence rates and surgical complications remained high following radical tumor resection. RT was effective for recurrent tumors and should be considered being the primary treatment for recurrences or difficult tumors, which are not amenable to total resections.