Abstract:Chordoma is a rare tumor derived from the residual tissue of the notochord, and the skull base chordoma accounts for about one third. Surgical treatment is the first choice for skull base chordoma, and the degree of surgical resection is highly correlated with prognosis. Because most of the tumors are epidural and locate on the ventral side of the skull base, transnasal endoscopic approach is suitable for tumor resection with little damage to brain tissue. Radiotherapy is an important adjuvant treatment for skull base chordoma. Postoperative radiotherapy applied to the patients with residual tumor can significantly prolong their progression free survival. Chordoma chemotherapy is still in clinical trials. Due to poor efficacy of traditional chemotherapy drugs for chordoma, new targeted therapic or immunotherapeutic drugs are needed to be developed. With the continuous advancement of comprehensive treatment technology, the therapeutic effect of skull base chordoma will be gradually improved.
