Presacral and postsacral extraspinal ependymoma. Report of a case and review of the literature.

A 29-year-old woman underwent surgery for an expanding subcutaneous postsacral mass. At surgery the mass was found to be an extraspinal ependymoma, completely separated from the central nervous system. The tumor was completely excised, with no evidence of recurrence 12 months postoperatively. Review of the literature reveals that only 17 cases of extraspinal postsacral ependymoma and 28 cases of presacral ependymoma have been reported. Postsacral tumors usually present as subcutaneous masses, but presacral tumors present with constipation, abdominal mass, neurologic deficit, and bony erosion. The treatment of choice for this tumor is wide local excision. Radiation may be of some value for local disease; chemotherapy has not been successful. Local recurrence has occurred in 23.5 per cent of the postsacral cases; 50 per cent of these patients died. The local recurrence rate is 14 per cent for patients with presacral masses, and the subsequent mortality rate has been 75 per cent. Distant metastases have occurred in three patients with postsacral tumors and in two patients with presacral tumors. The greatest chance for cure of this tumor lies with complete excision at the initial operation.