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. This evidence concerns the gene TERT and astrocytoma (excluding glioblastoma).