Below, the molecular consequences of key mutations/markers—MYCN (N-Myc), HOXB13, BRAF V600E, H3 K27M, TERT promoter, and TP53—in the pathogenesis of primary spinal cord tumors (ependymoma, astrocytoma, glioblastoma, and ganglioglioma) are reviewed. Here, MYCN is linked to glioblastoma.