CHEMOTHERAPEUTIC TREATMENT OF MALIGNANT CHORDOMA IN CHILDREN

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.