Chordoma and Notochord Tumors

Abstract

Notochord tumors present as malignant or benign neoplasms that are believed to arise from residual notochord cells. A chordoma is classically described as a malignant bone tumor theorized to originate from notochord cells and with a predilection for the sacrum, mobile spine, and base of the skull. The mainstay of treatment for chordoma is wide en bloc resection, often combined with adjuvant radiation therapy. The prognosis is poor because of the local aggressiveness of the tumor and its relative resistance to radiation and chemotherapy. Benign notochordal cell tumors also arise in the spine from residual notochord cells, but remain intraosseous and are not locally aggressive. Histologically, both malignant and benign notochordal cell tumors contain physaliferous cells, which have foamy, mucin-containing vacuoles, and some evidence suggests a continuum of disease from benign notochordal cell tumors to chordoma. In this article we describe the clinical, radiographic, and histologic evaluation of notochord tumors and their treatment, with a focus on tumors within the sacrum and coccyx.

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