期刊
BIOCHIMICA ET BIOPHYSICA ACTA-REVIEWS ON CANCER
卷 1877, 期 6, 页码 -出版社
ELSEVIER
DOI: 10.1016/j.bbcan.2022.188812
关键词
Chordoma; Modeling systems
资金
- Department of Orthopedic Surgery at the University of Miami Miller School of Medicine
- Sarcoma Foundation of America (SFA)
- Chordoma Foundation
This review summarizes recent enhancements and improvements made to generate useful chordoma models and discusses their applications in drug discovery and precision medicine.
Chordomas are malignant bone tumors that arise from remnants of the notochord. These tumors are generally slow-growing, locally aggressive, and invasive. Chordomas are typically resistant to conventional chemo-and radiotherapy. The clinical management of this disease is very challenging, usually, treatment is surgical resec-tion, which may be combined with radiotherapy. Although chordomas have undergone histologic and genetic analysis, the molecular mechanisms that drive their pathogenesis and resistance are still largely unknown. For many years this could be attributed to the lack of accurate and reliable in vitro and in vivo tumor models. Yet, over the past decade, many efforts have been made to prioritize the generation of useful chordoma cell lines, and tumor models that have shed more light on this malignancy and have made efficacious drug discovery a greater possibility. This review summarizes and discusses recent enhancements and improvements made to generate useful chordoma models and their applications in drug discovery and precision medicine.
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