Related Experiment Video
Updated: Oct 28, 2025
Endoscopic Cholesteatoma Surgery
Published on: January 19, 2022
Chordoma: Current status, problems, and future directions
Mary Frances Wedekind1, Brigitte C Widemann1, Gregory Cote2
1Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.
Abstract:
Chordoma is a rare tumor that occurs along the axial spine in pediatrics and adults, with an incidence of approximately 350 cases per year in the United States. While typically described as slow-growing, many patients will eventually develop loco-regional relapse or metastatic disease with few treatment options. Despite numerous efforts over the last 10+ years, effective treatments for patients are lacking. As subtypes of chordoma are identified and described in more detail, further knowledge regarding the natural history of each type, tumor location, age differences, genomic variability, and an overall better understanding of chordoma may be the key to developing meaningful clinical trials and effective therapies for patients with chordoma.
Insights
Chordoma, a rare axial spine tumor, often relapses or metastasizes despite slow growth, necessitating better understanding for effective chordoma treatments.
Area of Science:
- Oncology
- Genetics
Background:
- Chordoma is a rare tumor affecting the axial spine in both pediatric and adult populations.
- Despite a typically slow-growing nature, chordoma frequently leads to loco-regional relapse or metastatic disease.
- Current treatment options for chordoma remain limited, with a significant unmet need for effective therapies.
Purpose of the Study:
- To highlight the critical need for enhanced understanding of chordoma biology.
- To emphasize the potential of detailed subtype analysis for therapeutic development.
- To underscore the importance of natural history, genomic variability, and age-related differences in chordoma research.
Main Methods:
- Review of existing literature on chordoma incidence, natural history, and treatment outcomes.
- Analysis of the impact of tumor location, age, and genomic variability on chordoma progression.
- Identification of knowledge gaps in understanding chordoma subtypes.
Main Results:
- Chordoma presents a significant challenge due to its propensity for relapse and metastasis.
- Limited effective treatments exist, despite over a decade of research efforts.
- Subtyping chordoma reveals distinct characteristics influencing disease behavior.
Conclusions:
- A deeper understanding of chordoma subtypes, natural history, and genomic variability is crucial.
- This enhanced knowledge is essential for designing effective clinical trials and therapies for chordoma patients.
- Further research into chordoma's complexities may unlock new treatment avenues.
Related Concept Videos
02:07Tumor 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...
01:27Growth of Cartilage and Bone Tissue