Chordoma
ICD-10 C76.7 · ICD-11 2B5J&XA4S38

Treatment of Chordoma with Multinodular or Multifocal Local-Regional Relapse

This page addresses chordoma that has relapsed in a multinodular or multifocal local-regional pattern, as well as locally relapsed chordoma where neither surgery nor high-dose radiotherapy remains a feasible option.

In this setting, multifocal disease makes curative resection inappropriate. The clinical priority shifts to preventing the consequences of progression and preserving function — a cure is virtually impossible, and re-resection with curative intent should not be performed. Only limited resection aimed at preventing progression-related complications may be considered.

Management follows active palliative care principles. Depending on the individual clinical picture, a combination of loco-regional approaches and, where appropriate, systemic therapy may be considered — the full range of options and their sequencing is detailed in the complete protocol.

Primary clinical goal: Pain control and reduction of pain.

References

DOI: 10.1093/annonc/mdx054

  • Multinodular local-regional relapse. In cases of multifocal disease, a cure is virtually impossible so re-resection with curative intent should not be performed (IV-B); in these cases, only a limited resection should be considered with the goal of preventing the ill effects associated with disease progression whilst preserving function.
  • 'Salvage palliative/supportive treatment choices' include debulking surgery, low-dose RT, stereotactic body RT (SBRT), including radiosurgery to small volume, radiofrequency ablation (RFA) and other loco-regional approaches (i.e. cryotherapy), systemic therapy, PC and observation.
  • Medical therapy is an appropriate palliative option for patients whose disease is actively progressing or who are symptomatic.
  • Retrospective data suggest that cryoablation and RFA can be safe and useful palliative treatments in recurrent extracranial chordomas with a benefit in pain control.
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