Background. Dedifferentiated chordoma is an unusual and aggressive var
iant of chordoma which is likely to metastasize. Few reports exist of
treatment of these tumors with chemotherapy. Methods. In 1988, two pat
ients with dedifferentiated sacral chordomas were seen at the Universi
ty of Chicago Hospitals. Both developed metastatic disease less than a
year after sacral resection and radiation therapy. These patients' di
agnoses, courses, and treatments were reviewed along with the literatu
re on chemotherapy in both conventional and dedifferentiated chordomas
. Results. Both patients obtained complete remissions, one to a six-dr
ug regimen and the other to ifosfamide. Conclusions' A trial of reason
ably aggressive chemotherapy is warranted in patients with metastatic
dedifferentiated chordoma. The optimum regimen is unclear, but agents
active in high-grade sarcomas are logical choices.