Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients

Background. Chordomas are lobulated neoplasms composed of physaliphorous cells and their precursors; some have atypical, epithelioid, or spindle cell features. Fewer than one‐sixth of chordomas arise in the mobile (cervical, thoracic, or lumbar) spine. Forty‐eight percent originate in the sacrococcygeal region and 39% in the sphenoocciput. Methods. The study included 40 patients, 27 men and 13 women (2:1), with chordoma of the mobile spine. Their clinical and histopathologic features are described. Results. Nineteen tumors (48%) were located in the cervical spine, 7 (17%) in the thoracic spine, and 14 (35%) in the lumbar area. Most patients underwent subtotal removal of the tumor and postoperative irradiation. Variations in histologic appearance, including an occasional chondroid background, did not affect biologic behavior. Twenty‐three patients (58%) were alive 5 years after surgery. Eventually, 25 patients (63%) died of tumor. Metastasis developed in two patients (5%). In contrast to some other studies, metastasis was a rare occurrence. Conclusion. Chordoma of the mobile spine is a slow‐growing, recurring neoplasm of low metastatic potential that incapacitates by locally aggressive growth.