musculoskeletal and connective tissue disorders
Primary Malignant Bone Tumors
Chordoma
Chordoma, which is rare, develops from the remnants of the primitive notochord. It tends to occur at the ends of the spinal column, usually in the middle of the sacrum or near the base of the skull. A chordoma in the sacrococcygeal region causes nearly constant pain. A chordoma in the base of the skull can cause deficits in a cranial nerve, most commonly in nerves to the eye. Symptoms of chordoma may exist for months to several years before diagnosis. A chordoma appears on imaging studies as a destructive bone lesion that may be associated with a soft tissue mass. Biopsy is done. Chordomas in the sacrococcygeal region may be cured by radical en bloc excision. Chordomas in the base of the skull are usually inaccessible to surgery but may respond to radiation therapy. The rate of local recurrence is high after tumor resection. Metastasis, although less common, may occur. 