Surgical management of chordoma: A systematic review

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. Methods: We performed a systematic search using the keywords "chordoma" in combination with "surgery", "spine", "sacrum" and "radiotherapy". Results: Fifty-eight studies, describing 1359 patients with diagnosis of chordoma were retrieved. 17 studies were performed on subjects with cervical chordomas and 49 focused on patients with sacrococcygeal chordomas. The remaining studies included patients with chordomas in cranial region and/or mobile spine and/or sacroccygeal region. The recurrence rate ranged from 25% to 60% for cervical chordomas, and from 18% to 89% for sacrococcygeal chordomas. Conclusion: Despite the remarkable advances in the local management of chordoma performed in the last decades, the current results of surgery alone are still unsatisfactory. The radical en bloc excision of tumour is technically demanding, particularly in the cervical spine. 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.