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.

Natural history and surgical treatment of chordoma: a retrospective cohort study

“…[16][17][18][19][20] Some authors, such as York et al, 7 have reported prolonged progression-free survival with radiotherapy for patients with subtotal resection, whereas others have reported that this had little effect. Because of the small sample that underwent adjuvant radiotherapy in the present study, our report is too limited to formulate any conclusion.…”

Combined Anterior and Posterior En Bloc Vertebrectomy for Lumbar Chordoma

“…Chordoma is thought to be a primary bone tumor that occurs with a predilection for the clivus and sacrum 1,3,6,9) . This anatomical distribution supports the generally accepted theory that it arises from notochordal remnants.…”

“…So, it is gener-ally agreed that complete surgical excision of the tumor is the only curative procedure 8) . As has been recognized so far, recurrence rates after any treatments of chordomas have been high and unsatisfactory 1,3,4) . Surgical resection has become more aggressive in recent years evolving from intralesional debulking to total resection to reduce local recurrence rate 4) .…”

“…Thus, chordoma is thought to be a clinically malignant bone tumor 9) . Chordoma may occur anywhere along the spinal column, but it usually involves the sacrum or skull base presenting in late middle-age and older adults 3,7) . Chordomas of the vertebrae are known to be more locally aggressive, more likely to metastasize and have a poorer 5-year survival rate than sacral and clival lesions 8) .…”

A Case of Extraosseous Chordoma at the Cervico-Thoracic Junction

“…It is of notocordal origin and arises along the cranio-coccygeal axis. Chordoma accounts for approximately 5% of all malignant bony neoplasms [1]. About 50% arise from the sacrococcygeal region, 35% from the spheno-occipital region and 15% along the spinal axis [2].…”