OBJECTIVE

Chordomas are a rare and relatively slow-growing malignancy of notochordal origin with a nearly 50% recurrence rate. Chordomas of the cervical spine are particularly challenging tumors given surrounding vital anatomical structures. Although standard in other areas of the spine, en bloc resection of cervical chordomas is exceedingly difficult and carries the risk of significant postoperative morbidity. Here, the authors present their institutional experience with 13 patients treated with a structure-sparing radical resection and adjuvant radiation for cervical chordomas.

METHODS

Records of the standing senior author and institutional database of spinal surgeries were retrospectively reviewed for surgically managed cervical and high thoracic chordomas between 1997 and 2022. Chordomas whose epicenter was cervical but touched the clivus or had extension to the thoracic spine were included in this series. Clinical and operative data were gathered and analyzed for the index surgery and any revisions needed. Outcome metrics such as recurrence rates, complication rates, functional status, progression-free interval (PFI) and overall survival (OS) were evaluated.

RESULTS

The median patient age at diagnosis was 57 (range 32-80) years. The median modified Rankin Scale (mRS) score at the time of presentation was 1 (range 0-4). Approximately 40% of tumors were located in the upper cervical spine (occiput-C2). The median time from diagnosis to surgery was 74.5 (range 10-483) days. Gross-total resection was achieved in just under 40% of patients. All patients received adjuvant radiotherapy. The mean duration of follow-up was 4.09 years, with a mean PFI of 3.80 (range 1.16-13.1) years. Five patients experienced recurrence (38.5%). The mean OS was 3.44 years. Three patients died during the follow-up period; 2 due to disease progression and 1 died in the immediate postoperative period. One patient was lost to follow-up. A significant positive relationship was identified between high cervical tumor location and disease recurrence (p = 0.021).

CONCLUSIONS

While en bloc resection is appropriate and feasible for tumors in the sacral spine, the cervical region poses a significant technical challenge and is associated with increased postoperative morbidity. Radical resection may allow for achievement of negative operative margins and, along with sparing postoperative morbidity following resection of cervical chordomas, maintaining a similar rate of recurrence when compared with en bloc resection while preserving quality of life.