Is There a Role for Chemotherapy in Unresectable Ganglioneuroblastoma Intermixed?

Ganglioneuroblastoma intermixed (GNB-I) is a rare pediatric tumor with favorable outcomes when treated primarily with surgical resection. In patients for whom surgical intervention is not feasible, chemotherapy is often considered, although its efficacy remains controversial. This retrospective study examined 12 patients with nonmetastatic GNB-I at Phoenix Children's Hospital. Four patients received chemotherapy but showed no significant tumor reduction postchemotherapy, and all required surgery afterward. Chemotherapy led to notable toxicities, including febrile neutropenia and anaphylactic reaction. These findings suggest chemotherapy offers limited benefit while posing significant risks of cytotoxic adverse events for unresectable GNB-I, and surgery should be prioritized. Further longitudinal studies are needed to confirm these results.