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New Prospects for Molecular Targets for Chordomas

Mohammad Zeeshan Ozair1, Pavan Pinkesh Shah2, Dimitrios Mathios2

  • 1Laboratory of Stem Cell Biology and Molecular Embryology, The Rockefeller University, 1230 York Avenue, New York, NY 10065, USA.

Neurosurgery Clinics of North America
|March 10, 2020
PubMed

Insights

Chordomas are rare midline skeletal tumors. New molecular targeted therapies and immunotherapies show promise for managing these recurrent cancers by exploiting key molecular vulnerabilities.

Area of Science:

  • Oncology
  • Skeletal Tumors
  • Molecular Pathology

Background:

  • Chordomas are malignant, recurrent tumors originating from notochordal remnants along the midline skeleton.
  • Effective systemic therapy is crucial for managing advanced and recurrent chordoma.
  • Understanding chordoma pathogenesis is key to developing novel treatment strategies.

Purpose of the Study:

  • To review the major molecular pathways implicated in chordoma pathogenesis.
  • To highlight molecular vulnerabilities that can be exploited for future therapies.
  • To discuss the potential of targeted therapies and immunotherapies for chordoma.

Main Methods:

  • Literature review of studies on chordoma pathogenesis and treatment.
  • Analysis of molecular pathways involved in chordoma development.
  • Summary of ongoing clinical trials for targeted therapies and immunotherapies.

Main Results:

  • Several molecular pathways are implicated in chordoma development.
  • Molecular targeted therapies show promise in ongoing trials for mutated pathways.
  • Immunotherapies, including brachyury vaccination and checkpoint inhibition, yield encouraging results.

Conclusions:

  • Chordoma pathogenesis involves specific molecular pathways that present therapeutic vulnerabilities.
  • Targeted therapies and immunotherapies represent promising avenues for chordoma treatment.
  • Further research into molecular vulnerabilities will drive the development of effective systemic therapies for chordoma.
Keywords:
BrachyuryChordomaImmunotherapyMolecular targeted therapyNotochordReceptor tyrosine kinase (RTKs)

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