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PHOX2B is a homeodomain transcription factor that is mainly expressed in neuronal cells of the peripheral autonomic nervous system, and plays a key role in the differentiation of neurons and chromaffin cells, in particular. PHOX2B has high sensitivity and specificity in neuroblastoma tissues. In addition, it is directly related to autonomic nervous system diseases that may occur with neuroblastoma （such as congenital megacolon and congenital central hypoventilation syndrome）.