Introduction

First described in 1864 by the German physician Rudolf Virchow, and later named in 1910 by James Homer Wright, neuroblastoma (NB) is a small round blue cell tumor that has the unique ability to mature and regress to complete resolution, either spontaneously or in response to treatment, or become undifferentiated and highly malignant leading to disease progression and ultimately death. Due to the diverse nature of NB, treatment ranges from simple observation in select infants to aggressive multimodal therapy, including myeloablative therapy with stem cell rescue and immunotherapy. Surgical resection continues to play an important role in the local control of NB for the majority of patients. Tumor staging and risk classification for NB have been refined as new genomic prognostic markers are identified, and efforts continue to unify risk-based treatment strategies worldwide. Survival is high for patients with low and intermediate-risk NB; therefore, efforts are focused on therapy reductions to diminish treatment-related complications. Unfortunately, most patients present with high-risk disease, where outcomes have improved but remain poor overall.

Content on this topic is referenced in SCORE Neuroblastoma overview