Neuroblastoma: A tumour with many faces

Summary 

Neuroblastoma is the most common malignant extracranial solid tumour in childhood. The prognosis of children with this malignancy varies from excellent to very poor, depending on age, staging and cytogenetic make up. These tumours originate from the neural crest and show a wide range of neuronal differentiation and malignant potential. Neuroblastomas are unique because of their ability to undergo differentiation and transformation to more benign tumours even with an extensive tumour mass. Advances in tumour genetics has led to a better understanding of the biology of neuroblastomas. This allows tailoring of therapy, reserving very intense treatment strategies for children with high-risk disease. Amplification of the oncogene MYCN remains the single most important cytogenetic marker presently. However, several other markers gain importance in the disease. Neuroblastomas can present with a wide variety of clinical signs and symptoms depending on the location of the primary tumour. In infants, the disease can manifest as acute respiratory or hepatic decompensation secondary to rapidly increasing size of the tumour or infiltration of the liver by tumour cells. Paraneoplastic phenomena like opsomyoclonus are described. Intensification of chemotherapy in high-risk cases using myeloablative regimes and autologous stem cell rescue improved the outcome of these children significantly. Novel treatment approaches using differentiation therapy and tumour-directed antibodies are incorporated in multimodality high-risk treatment regimes. Research to find more tumour specific therapies with fewer side effects is ongoing.

Keywords: Neuroblastoma, Children