The Cell Biology of Neuroblastoma

Abstract

Neuroblastoma is the third most common solid tumor in children and is the most common extracranial solid tumor in children (Maris, 2010; Coulter et al., 2008; Wickstrom et al., 2007). It is highly malignant and has a poor outcome despite aggressive treatment, even with autologous stem cell transplantation (Sartelet et al., 2008). It is a disease of the neural crest and the sympathetic nervous system, and most often originates from the adrenal gland. Neuroblastoma occurs as low risk, medium risk, and high risk forms, with the high risk form being difficult to treat even with the most advanced protocols. The prognosis for neuroblastoma is poor in advanced stages of the disease. Low risk forms may regress spontaneously, and may require no surgery and only observation (Kitanaka et al., 2002). Spontaneous remission occurs more commonly in neuroblastoma than in other cancers (Carlsen, 1990; Hero et al., 2008). The reasons for this are not at present understood. The overall cure rate is 40%, which indicates that new therapeutic strategies are needed (Sartelet et al., 2008). This chapter will focus on the cell and molecular biology of neuroblastoma, and the development of therapeutic strategies for its treatment. It will emphasize biologically unfavorable and clinically aggressive neuroblastomas rather than biologically favorable neuroblastomas.