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

Abstract

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.