Neuroblastoma: a master of disguise and a challenge to cure

Neuroblastoma is one of the commonest childhood cancers and typically affects very young children. It is characterized by a very broad spectrum of clinical presentation and outcome, driven by the biology of the tumour. This ranges from ‘low risk’ tumours, most commonly found in infants, which may spontaneously regress and have an excellent prognosis with minimal or no treatment, to ‘high risk’ disease, which carries a poor prognosis despite intensive multi-modal treatment. Although particular presentations may be associated with favourable or unfavourable outcome, the clinical features may mask the underlying biology of the tumour and a full assessment of the clinical and biological features is required to determine appropriate treatment. The International Neuroblastoma Risk Group classification, based on the age of the patient and the stage, grade and genetics of the tumour, is used to stratify treatment according to risk factors. This review provides an overview of current neuroblastoma management, focusing on how classification is applied in practice, and how this is used to determine individual patient treatment. The challenges that remain in treating patients with high-risk disease are discussed