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Chordoma is a rare bone cancer with a relatively poor long-term outcome. The tumor originates from fetal notochord and mainly occurs in the sacrum, skull base, and mobile spine. Tumor metastases are found in up to 40% of cases. Surgical treatment is usually the primary treatment, however, gross total ...

Chordoma is a rare bone cancer with a relatively poor long-term outcome. The tumor originates from fetal notochord and mainly occurs in the sacrum, skull base, and mobile spine. Tumor metastases are found in up to 40% of cases. Surgical treatment is usually the primary treatment, however, gross total resection is challenging. Adjuvant radiotherapy is recommended for all cases, although with limited evidence for success. Despite extensive translational research having improved patients’ outcomes, tumor recurrence is still common, regardless of resection rates and radiation types. Further, precision medicine based on molecular targets for chordoma is still in its infancy.

Nevertheless, understanding and treatment of chordoma have achieved great advancement in recent twenty years. For example, recent genomic and epigenomic studies have shed light on chordoma biology, which may lead to the development of novel treatment options. In addition, endoscopic endonasal approaches and high-dose particle radiation with low side effects have been increasingly applied by skull-base surgeons and radiologists, which have substantially increased patients’ outcomes.

The goal of this Research Topic is to describe recent progress in basic research and clinical management of chordoma. Topics of interest include, but are not limited to, surgical and radiation therapy, targeted therapy or immunotherapy, and basic research that will improve the understanding and treatment of chordoma of any body site.

• Surgical treatment strategies of chordoma (surgical technique, complications, follow-up et. al.)
• Benefits and side-effects of different radiation therapy types in chordoma.
• Targeted drugs for different molecular targets in chordoma
• Translational research on chordoma from basic research findings to the clinical level
• Genetic and epigenetic analyses that may help understand chordoma biology

Please note: manuscripts consisting solely of bioinformatics or computational analysis of public genomic or transcriptomic databases which are not accompanied by validation (independent cohort or biological validation in vitro or in vivo) are out of scope for this section and will not be accepted as part of this Research Topic.

Keywords: Chordoma, biology, radiotherapy, medical treatment, surgical treatment


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