title: Chemotherapeutic treatment of malignant chordoma in children.
abstract: OBJECTIVE
  We describe the effect of multiagent chemotherapy for malignant chordoma. Previous reports of other patients with malignant chordoma treated with chemotherapy as well as other therapeutic interventions are reviewed.
  
  
  METHODS
  We describe a 19-month-old girl with unresectable cervical chordoma metastatic to the lungs at diagnosis treated with multiagent systemic chemotherapy. CNS disease was diagnosed after one course of therapy, and intrathecal chemotherapy was then administered.
  
  
  CONCLUSIONS
  Ifosfamide and doxorubicin were efficacious in a patient with advanced metastatic disease, producing significant 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 the combination of cisplatin and 5-fluorouracil or high-dose methotrexate.
authors.affiliation: Division of Pediatric Hematology/Oncology, North Shore University Hospital/Cornell University Medical College, Manhasset, NY 11030, USA.
authors.forename: P G
authors.initials: PG
authors.lastname: Scimeca
authors.forename: A G
authors.initials: AG
authors.lastname: James-Herry
authors.forename: K S
authors.initials: KS
authors.lastname: Black
authors.forename: E
authors.initials: E
authors.lastname: Kahn
authors.forename: M E
authors.initials: ME
authors.lastname: Weinblatt
mesh_terms: D000328:Adult; D000493:Allopurinol / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002675:Child, Preschool; D002817:Chordoma / Q000188:drug therapy*; D003907:Dexamethasone / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006258:Head and Neck Neoplasms / Q000188:drug therapy*; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male
chemical_list: D000493:Allopurinol; D003907:Dexamethasone; D004317:Doxorubicin; D007069:Ifosfamide