The journal of spinal cord medicine
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Context: Chordomas are rare primary tumors of bone characterized by local aggressiveness and poor prognosis. The surgical exeresis plays a critical role for their management. Objective: The aim was to provide an overview of the surgical management of chordomas of the mobile spine and sacrum, describing the most common surgical approaches, the role of surgical margins, the difficulties of en block resection, the outcomes of surgery, the recurrence rate and the use of associated therapies. ⋯ Although radical surgery must still be considered the gold standard for the management of chordomas, a multidisciplinary approach is required to improve the local control of the disease in patients who undergo both radical and non-radical surgery. Adjuvant radiation therapy increases the continuous disease-free survival and the local recurrence-free survival. Level of evidence: Systematic review; level III.