Due to their origin in the notochord, chordomas occur in the mid-line of the axial skeleton. One half of cases occur in the sacrococcygeal region and one third occur at the base of the skull. Other rare sites include transverse processes of vertebrae and the paranasal sinuses. The clinical presentation depends on the location of the tumor. Sacrococcygeal tumors often present as low back pain with no characteristic pattern or time course.
Sacrococcygeal tumors can also present as bowel and bladder dysfunction.
Presacral tumors can sometimes be palpated on rectal exam. Sacral tumors are often large at presentation as a large volume of tumor can be accommodated within the pelvis. Anterior cervical tumors can present as dysphagia and posterior cervical tumors can cause neurological deficits. Tumors at the base of the skull may present with headaches. There is often an accompanying soft tissue mass.