In NB cells, PHOX2B and MSX1 expression are inversely correlated; such am observation is in accordance with the role of MSX1 in the activation of the NOTCH3 expression; in particular, NOTCH 3 is highly expressed in benign ganglioneuroma but lowly expressed in severe neuroblastoma, the latter being characterized by high PHOX2B expression [217]. Here, NOTCH3 is linked to neuroblastoma.