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 radical resection, the use of three-dimensional conformal radiotherapy or fractioned 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.