Sacrococcygeal chordoma: Report of a case and review of the literature.

A review of 222 patients with sacrococcygeal chordoma from the English language literature since the first American case in 1913 reveals that men are affected twice as frequently as are women after age 40, but not before. A history of prior trauma can be found in about 20 percent of patients, but no clear etiologic relation can be proved. The evidence for a congenital origin from displaced notochordal tissue is equally inconclusive. Metastasis occurs in only about 10 percent of patients, but recurrence of the primary lesion is almost universal. Of 67 patients followed, 11 survived 10 years or longer after diagnosis of chordoma. Resection of the tumor rarely is complete. Preservation of sacral stability and of sacral nerve pathways to the rectum and the bladder limit the extent of permissable surgery. Repeated local excision of the tumor recurring after initial excision and irradiation remains the treatment of choice.