UPDATES IN THE MANAGEMENT OF NEUROBLASTOMA

Abstract

Through international collaboration we are at the start of a new age in the management of this enigmatic tumour. Tumours are now grouped at diagnosis using the International Neuroblastoma Risk Grouping (INRG), which uses information from tumour tissue reflecting tumour biology, as well as radiology to define Image-defined risk factors (IDRF). Tumours in high risk groups receive maximal therapy in an attempt to try and improve outcomes which are still poor. Intermediate risk tumours, which have better outcomes due to better response to current therapy, are treated aggressively with combination therapies with proven therapeutic effects, but with increasing attention to the minimising adverse treatment effects. The treatment of low risk tumours is now vastly reduced, acknowledging the excellent outcome in these children using minimal therapy. It has become apparent that the consequences of therapy in these children can easily be worse than those from the tumour itself. For very low risk tumours trials are exploring the outcomes without any treatment. INRG allows more precise comparison of results between different international groups, and provides a template into which new prognostic variables can be introduced, and their value assessed. In many centres multi-array analysis is studying the genetic profile of each tumour; perhaps this will allow increasing individualisation of treatment programmes in the near future.

Key words: Neuroblastoma, International Neuroblastoma Risk Grouping, Image defined risk factors.