[High-risk neuroblastoma treatment strategy: The experience of the SIOPEN group]

Abstract

High-risk neuroblastoma comprises nearly half of cases of neuroblastoma and the long-term survival is less than 50% despite complex and intensive treatments. Studies conducted in Europe and in North America in the last two decades have identified a strategy based on four therapeutic phases: an intensive induction therapy, a local control by surgery and radiation, a consolidation phase with single or tandem high dose chemotherapy and autologous transplant, and immunotherapy to eliminate residual disease. Future treatment improvements are based on progress at each of these therapeutic steps and ultimately a better stratification of the strategy adapted to the type of risk. A more extensive tumor molecular profiling at diagnosis and relapse will help to develop new therapeutics and to guide risk-based strategies. Earlier use of immunotherapy and identification of more effective combinations in induction or in maintenance treatment, identification of indications of more intense consolidations using high-dose chemotherapy combined or not with metabolic irradiation by 131I-MIBG and the introduction of other targeted treatments are tracks being explored.

Keywords: Anti-GD2; Autogreffe de cellules souches hématopoïétiques; Autologous stem cell transplantation; Chimiothérapie à hautes doses; High dose chemotherapy; High risk neuroblastoma; Immunotherapy, Anti-GD2; Immunothérapie; Neuroblastome de haut risque.