Related Experiment Video
Updated: May 10, 2026

Role of Diffusion MRI Tractography in Endoscopic Endonasal Skull Base Surgery
Published on: July 5, 2021
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.
Background:
Chordomas of the skull base are rare locally aggressive neoplasms with a predilection for encapsulating critical neurovascular structures, bony destruction and irregular growth patterns, and from which patients succumb to recurrence and treatment failures.
Methods:
A review of the medical literature is performed, using standard search engines and identifying articles related to skull base chordomas, surgery, radiation therapy, chemotherapy, molecular genetics, and prospective trials.
Results:
A synthesis of the literature is presented, including sections on pathology, treatment, molecular genetics, challenges, and future directions.
Conclusion:
Beyond an understanding of the current treatment paradigms for skull base chordomas, the reader gains insight into the collaborative approach applied to orphan diseases, of which chordomas is a prime exemplar.
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.
Related Concept Videos
Targeted Cancer Therapies
There are several types of targeted therapies against specific...
Tumor Progression
Colon cancer is one of the best-documented examples of tumor progression. Early mutation in the APC gene in colon cells causes a small growth on the colon wall called a polyp. With time, this polyp grows into a benign, pre-cancerous tumor. Further...

