Management strategies for recurrent pediatric craniopharyngioma: new recommendations

OBJECTIVE The goal of this study was to identify the independent risk factors for recurrence or progression of pediatric craniopharyngioma and to establish predictors of the appropriate timing of intervention and best management strategy in the setting of recurrence/progression, with the aim of optimizing tumor control. METHODS This is a retrospective cohort study of all pediatric patients with craniopharyngioma who were diagnosed and treated at Boston Children’s Hospital between 1990 and 2017. This study was approved by the institutional review board at Boston Children’s Hospital. All statistical analyses were performed using Stata software. RESULTS Eighty patients (43 males and 37 females) fulfilled the inclusion criteria. The mean age at the time of diagnosis was 8.6 ± 4.4 years (range 1.2–19.7 years). The mean follow-up was 10.9 ± 6.5 years (range 1.3–24.6 years). Overall, 30/80 (37.5%) patients developed recurrence/progression. The median latency to recurrence/progression was 12.75 months (range 3–108 months). Subtotal resection with no adjuvant radiotherapy (p < 0.001) and fine calcifications (p = 0.008) are independent risk factors for recurrence/progression. An increase (%) in the maximum dimension of the tumor at the time of recurrence/progression was considered a statistically significant predictor of the appropriate timing of intervention. CONCLUSIONS Based on the identified independent risk factors for tumor recurrence/progression and the predictors of appropriate timing of intervention in the setting of recurrence/progression, the authors propose an algorithm for optimal management of recurrent pediatric craniopharyngioma to increase the likelihood of tumor control.