Converging paths to progress for skull base chordoma: Review of current therapy and future molecular targets

Salvatore Di Maio1, Esther Kong, Stephen Yip

  • 1Division of Neurosurgery, McGill University, Jewish General Hospital, Montreal, QC, Canada.

Abstract

Insights

Skull base chordomas are aggressive tumors that are difficult to treat. This review synthesizes current knowledge on their pathology, treatment, and genetics, offering insights into managing this rare cancer.

Area of Science:

  • Oncology
  • Neurosurgery
  • Genetics

Background:

  • Skull base chordomas are rare, locally aggressive neoplasms.
  • These tumors encapsulate critical neurovascular structures and cause bony destruction.
  • Recurrence and treatment failures are common challenges.

Purpose of the Study:

  • To provide a comprehensive overview of skull base chordomas.
  • To synthesize current knowledge on pathology, treatment, and genetics.
  • To highlight challenges and future directions in managing these rare tumors.

Main Methods:

  • Systematic review of the medical literature.
  • Utilized standard search engines for relevant articles.
  • Focused on skull base chordomas, surgery, radiation, chemotherapy, and genetics.

Main Results:

  • Presented a synthesis of current literature on skull base chordomas.
  • Detailed information on pathology, treatment modalities, and molecular genetics.
  • Identified key challenges and outlined future research directions.

Conclusions:

  • Enhanced understanding of current treatment paradigms for skull base chordomas.
  • Illustrates the collaborative approach needed for rare (orphan) diseases.
  • Chordomas serve as a prime example of challenges in rare cancer management.