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Updated: May 28, 2026
Role of Diffusion MRI Tractography in Endoscopic Endonasal Skull Base Surgery
Published on: July 5, 2021
Current therapeutic options and novel molecular markers in skull base chordomas
Filippo Gagliardi1, Nicola Boari, Paola Riva
1Department of Neurosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy. gagliardi.filippo@hsr.it
Abstract:
Chordomas are extremely rare tumours. They arise in the spheno-occipital region in 35% of cases. Chordomas usually present benign histopathological features but often exhibit a malignant clinical behaviour. Radical surgical removal and high-dose radiation therapy seem to be effective in tumour control and to improve survival rate. Despite the advancements in microsurgical techniques and the development of radiation therapies, clival chordomas still represent a challenge. Nevertheless it appears that chordomas that have been resected to the same extent and that received post-operative radiotherapy might exhibit different rates of regrowth. This result supports the hypothesis that the recurrence rate of chordomas might be dependent on biological variables other than the extent of resection and the post-operative radiotherapy. Genetic and molecular studies on oncogenesis of chordomas are still limited, but they represent the basis for the development of molecular targeted therapies. We present a review of the current knowledge about skull base chordomas biology, therapeutic options and related clinical outcome.
Insights
Chordomas, rare skull base tumors, may recur due to biological factors beyond surgery and radiation. Further research into chordoma biology could enable targeted therapies.
Area of Science:
- Oncology
- Neurosurgery
- Genetics
Background:
- Chordomas are rare tumors originating in the spheno-occipital region, often presenting benign histology but malignant behavior.
- Current treatments include radical surgery and high-dose radiation, yet clival chordomas remain challenging.
- Recurrence rates may depend on factors beyond surgical extent and radiotherapy.
Purpose of the Study:
- To review current knowledge on skull base chordoma biology.
- To discuss therapeutic options and clinical outcomes.
- To explore the potential for molecular targeted therapies based on oncogenesis studies.
Main Methods:
- Review of existing literature on chordoma biology, treatment, and outcomes.
- Analysis of factors influencing tumor control and survival rates.
- Examination of the hypothesis that biological variables impact chordoma recurrence.
Main Results:
- Despite aggressive treatment, clival chordomas present significant challenges.
- Tumor regrowth rates may vary even with similar resection extent and radiotherapy.
- This suggests biological factors play a crucial role in chordoma recurrence.
Conclusions:
- Chordoma recurrence may be influenced by intrinsic biological variables.
- Limited genetic and molecular studies highlight the need for further research.
- Understanding chordoma oncogenesis is essential for developing targeted therapies.
