From sympathetic neuron development to neuroblastoma

Neuroblastoma (NB) is a childhood tumor that arises from the sympathoadrenal lineage. The mechanisms that direct sympathetic neuron generation are fundamentally different from the control of neurogenesis in other parts of the nervous system and involve the transcription factor Phox2b and the tyrosine kinase receptor Alk. Mutations in Phox2b and Alk predispose to NB in familiar forms of this disease. The expression of mutant Phox2b and Alk in embryonic sympathetic ganglion cells identified signaling pathways that result in aberrant growth and may contribute to NB predisposition.