Purpose: We describe the effect of multiagent chemotherapy for maligna
nt chordoma. Previous reports of other patients with malignant chordom
a treated with chemotherapy as well as other therapeutic interventions
are reviewed. Patients and Methods: We describe a 19-month-old girl w
ith unresectable cervical chordoma metastatic to the lungs at diagnosi
s treated with multiagent systemic chemotherapy. CNS disease was diagn
osed after one course of therapy, and intrathecal chemotherapy was the
n administered. Conclusions: Ifosfamide and doxorubicin were efficacio
us in a patient with advanced metastatic disease, producing significan
t disease regression. The addition of intrathecal or intraventricular
therapy with hydrocortisone, ARA-C, and methotrexate was effective in
controlling CNS disease due to chordoma. There was no apparent benefit
from the use of actinomycin-D, cyclophosphamide and vincristine nor t
he combination of cisplatin and 5-fluorouracil or high-dose methotrexa
te.