Chordoma: a 20-year clinicopathologic review of the experience at Groote Schuur Hospital, Cape Town.

Eighteen chordomas (11 females and 7 males) were seen over a 20-year period; 61% of the tumors occurred in the sphenoid region. The youngest patient was 3 years old and had a family history of chordoma. Histologically, the tumors were divided into classical (epithelial) and chondroid variants, which in this series, had no effect on outcome. Follow-up on 12 patients ranged from 3 to 170 months, and they were treated with various combinations of surgery and radiotherapy. One patient was administered the neutron beam and died after 94 months. The mean survival of this series is 73.4 months, with a survival rate of 50% (6 out of 12 patients alive). Surgical resection offers the best chance of survival, but chordomas have a propensity to metastasize, hence have a poor prognosis.