Abstract
Chordoma is a neoplasm that arises from noto-chordal remnants in the nucleus pulposus of the intervertebral discs, designated as normally situated chordal vestiges and/or from abnormal ectopic remnants, because they are located where notochord should have regressed, i.e., in the spheno-occipital region (including clivai ecchordosis), in the posterior part of nasopharynx, in the substance of bone in the vertebral bodies, or in the tissues surrounding them. Chordoma, therefore, occurs almost exclusively in the midline of the axial skeleton, with a distinct predilection for the distal and proximal ends of the vertebral columna, i.e., the spheno-occipital and sacrococcygeal regions.
Definition, WHO: “A malignant tumor characterized by a lobular arrangement of tissue which is usually made up of highly vacuolated cells (“physaliphorous cells”) and mucoid intercellular material. These tumors are restricted to the axial skeleton.”
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Schajowicz, F. (1981). Other Tumors. In: Tumors and Tumorlike Lesions of Bone and Joints. Springer, New York, NY. https://doi.org/10.1007/978-1-4684-0098-4_8
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