title: Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases.
abstract: BACKGROUND
  Dedifferentiated chordoma is an unusual and aggressive variant of chordoma which is likely to metastasize. Few reports exist of treatment of these tumors with chemotherapy.
  
  
  METHODS
  In 1988, two patients with dedifferentiated sacral chordomas were seen at the University of Chicago Hospitals. Both developed metastatic disease less than a year after sacral resection and radiation therapy. These patients' diagnoses, courses, and treatments were reviewed along with the literature on chemotherapy in both conventional and dedifferentiated chordomas.
  
  
  RESULTS
  Both patients obtained complete remissions, one to a six-drug regimen and the other to ifosfamide.
  
  
  CONCLUSIONS
  A trial of reasonably 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.
authors.affiliation: Department of Medicine, University of Chicago Hospitals, IL 60637-1470.
authors.forename: G F
authors.initials: GF
authors.lastname: Fleming
authors.forename: P S
authors.initials: PS
authors.lastname: Heimann
authors.forename: J K
authors.initials: JK
authors.lastname: Stephens
authors.forename: M A
authors.initials: MA
authors.lastname: Simon
authors.forename: M K
authors.initials: MK
authors.lastname: Ferguson
authors.forename: R S
authors.initials: RS
authors.lastname: Benjamin
authors.forename: B L
authors.initials: BL
authors.lastname: Samuels
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000473:pathology; D002817:Chordoma / Q000188:drug therapy* / Q000473:pathology / Q000556:secondary; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000556:secondary; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D012447:Sacrum