Neuroblastoma

Neuroblastoma originates from primordial neural crest cells that normally give rise to adrenal medulla and sympathetic ganglia. While it accounts for 6% of all childhood malignancies, neuroblastoma accounts for 15% of childhood cancer mortality. Neuroblastoma is a disease of early childhood, though this tumor is occasionally seen in adolescents and young adults. Neuroblastoma usually presents with an adrenal mass or a tumor arising along the sympathetic neural chain. The most common presenting feature is an asymptomatic abdominal mass, with metastases detected at the time of diagnosis in 75% of cases. Biologic features of neuroblastoma tumors are of critical importance for risk assessment. Amplification of MYCN is strongly correlated with advanced-stage disease and treatment failure. Other key prognostic factors include age at diagnosis, disease stage, tumor histology, tumor cell ploidy, and the status of specific chromosomal segments. Modern treatment regimens are risk-based. Reduction in toxicity is a major goal in current trials for patients with nonhigh-risk neuroblastoma. Integration of multiple treatment modalities with targeted therapeutics is being studied in the setting of high-risk disease.