Chordomas.

Chordomas constitute between 1% and 4% of primary malignant bone tumors. Approximately 50% originate in the sacrum, 35% at the base of the skull, and 15% in the true vertebrae. The majority of tumors are encountered from the fifth through seventh decades, with a male preponderance. The clinical symptoms and signs are nonspecific and lead to frequent errors in clinical diagnosis. Radiographic findings include nonspecific destruction of the vertebral body, with reactive sclerosis; calcification is more often seen in sacral tumors. Computed tomography (CT) frequently reveals an anterolateral soft-tissue mass, which is often more extensive than the osseous involvement. The optimal treatment for sacral tumors should be en bloc resection of the tumor performed through intact bone a level above (wide local excision); for vertebral lesions, an anterior surgical approach with resection of the vertebral body should be performed. While conventional radiation has little efficacy in this tumor, a variety of innovative newer approaches may prove more effective in the future. Conventional chemotherapy has not proved effective in this tumor. With early diagnosis, and more effective surgical therapy, the current disease-free survival at five years should be between 30% and 50%.