Neuroblastoma

Excerpt

Neuroblastoma (NB) is the most frequently-occurring extracranial childhood tumor. It is classified as an embryonal neuroendocrine tumor, originating from neural crest progenitor cells. Hence, it can occur anywhere along the sympathetic nervous system, including the superior cervical, paraspinal, and celiac ganglia; the majority arise in the adrenal glands. Due to the high variability in its presentation, clinical signs and symptoms at presentation can range from a benign palpable mass with distension to major illness from substantial tumor spread. Although overall increases in five-year event-free survival have been reported, subgroup-specific analysis of mortality has revealed discordance between the high cure rates for the more benign low-risk forms and little improvement in the high-risk groups. Thus, the impetus for the development of targeted therapeutics in the intensive management of high-risk groups is strong.