Little is known about the genetic events responsible for driving the growth of chordomas, but it is well established that dysregulation of different signaling pathways as canonical as BMP4/SMAD and RAS/RAF/MEK/ERK are involved in the development of these tumors, while others like PI3K/AKT/mTOR are starting to be explored in this context (89–93). Here, MTOR is linked to chordoma.