Clinical outcome in children with recurrent craniopharyngioma after primary surgery

PURPOSE: The purpose of this article is to report the clinical outcome in children with recurrent craniopharyngioma after primary surgery. PATIENT AND MATERIALS: Fourteen children with craniopharyngioma treated with primary surgery developed local recurrence. The median time to recurrence from primary surgery was 19 months (range, 2-156 months). At first recurrence (n = 14), seven children (50%) underwent reoperation. Five children (36%) received radiotherapy, and two children did not undergo any treatment. At second recurrence (n = 7), six children underwent radiotherapy (86%), and one had surgery. External-beam radiation was delivered with 6-MV or 10-MV x-rays by use of three-dimensional conformal technique (n = 4) or fractionated stereotactic radiotherapy (n = 7) using the Laitinen stereoadapter. Total dose ranged from 54 to 55.8 Gy at 1.8 Gy/fraction. RESULTS: The median follow-up from primary surgery is 8.5 years (range, 3-15.8 years). The 5-, 10-, and 15-year overall survival was 100%, 86%, and 86%, respectively, and the disease-free survival was 92%, 60%, and 60%, respectively. The median follow-up from date of first relapse was 6 years (range, 2.5-10 years). After treatment for first recurrence, the 2- and 5-year second-recurrence-free survival was 71% and 29%, respectively. After radiotherapy, the 2- and 5-year second-recurrence-free survival was 100% and 100%, respectively, compared with 43% and 0%, respectively, for surgery alone. Univariate analysis revealed significantly superior local control with radiotherapy compared with surgery. The local control rate at last follow-up, after stratifying for treatment at first and second recurrence, were analyzed at first and second relapse was 90% and 0% after radiotherapy and surgery, respectively. If radiotherapy was used for first or second recurrence, the 5-, 10-, and 15-year relapse-free survival was 100%, 83%, and 83%, respectively, compared with 67%, 0%, and 0%, respectively, for surgery alone. The median time to second relapse after surgery for first relapse was 12 months (range, 2-36 months). After primary surgery, all 14 children developed panhypopituitarism, requiring lifelong hormone supplementation. After surgery at recurrence, three children (3/7, 43%) experienced intraoperative bleeding, resulting in permanent neurologic deficits in two. No child has shown any signs of radiation-induced optic neuropathy. DISCUSSION: In children with recurrent craniopharyngioma after primary radical resection, the use of three-dimensional conformal radiotherapy or fractionated stereotactic radiotherapy results in very good local control with a low incidence of complications. In young children with stable tiny recurrences, a policy of close surveillance could be adopted for the brain to mature before beginning radiotherapy. The use of secondary surgery for recurrent tumors is associated with a low cure rate and a high risk of complications.