Primary chordomas of the cervical spine: a consecutive series of 14 surgically managed cases

Abstract

Object Cervical chordomas are rare lesions and usually bring about challenges in treatment planning because of their wide extension and complicated adjacent anatomy. There are few large published series at present focusing on cervical chordomas. The authors studied a consecutive series of 14 patients with primary cervical chordomas who underwent surgical treatment and were observed between 1989 and 2008. By reviewing the clinical patterns and follow-up data, they sought to investigate the clinical characters, tailor the appropriate surgical techniques, and establish prognosis factors for cervical chordomas. Methods Hospitalization and follow-up data in the 14 patients were collected. All patients underwent piecemeal tumor excision and reconstruction for stability; total spondylectomy was achieved in 5 cases. Postoperative radiotherapy was administered in all patients. Kaplan-Meier plots were used to represent tumor recurrence and patient survival, and log-rank testing was used to determine the risk factors of local recurrence. Results Follow-up ranged from 8 to 120 months (mean 58.6 months). Symptom and neural status in most patients improved after surgery. The 1- and 5-year disease-free survival rates were 78.6% and 50%, respectively, and the 1- and 5-year survival rates were 92.9% and 85.7%, respectively. Log-rank tests revealed that the following variables were significantly associated with a high rate of tumor recurrence: age less than 40 years or greater than 70 years (p = 0.006) and an upper cervical tumor location (p = 0.019). Conclusions Chordomas in the cervical spine are usually neoplasms that exhibit insidious growth and a wide extension by the time of diagnosis. Radical intralesional debulking surgery and postoperative radiotherapy have been effective treatment. A limited application of en bloc tumor resection and the highly likely intraoperative intralesional tumor seeding may partially explain the high local recurrence rate, whereas the chance of distant metastases, fortunately, is very low. Most recurrence were documented within 3 years. Some specific surgical techniques should be emphasized to minimize tumor seeding. Patients with upper cervical chordomas, younger adults, and elderly adults have worse prognosis. For patients with chordoma extending to both the anterior and posterior spinal columns, total spondylectomy combined with piecemeal excision is recommended for a better prognosis.