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Review

Immunologic and Targeted Molecular Therapies for Chordomas: A Narrative Review

1
Department of Orthopaedic Surgery, Montefiore Einstein, Bronx, NY 10461, USA
2
Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA
3
Department of Neurological Surgery, Montefiore Einstein, Bronx, NY 10461, USA
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2024, 13(19), 5679; https://doi.org/10.3390/jcm13195679
Submission received: 4 September 2024 / Revised: 17 September 2024 / Accepted: 20 September 2024 / Published: 24 September 2024
(This article belongs to the Section Orthopedics)

Abstract

Chordomas are rare sarcomas arising from notochordal tissue and occur most commonly in the spine. The standard of care for chordomas without evidence of metastatic disease generally consists of en bloc resection followed by adjuvant radiotherapy. However, long-term (20-year) survival rates are approximately 30%. Chordomas are generally considered as chemo resistant. Therefore, systemic therapies have rarely been employed. Novel immunotherapies, including antibody therapy and tumor vaccines, have shown promise in early trials, leading to extended progression-free survival and symptom relief. However, the outcomes of larger trials using these vectors are heterogeneous. The aim of this review is to summarize novel chordoma treatments in immune-targeted therapies. The current merits, trial outcomes, and toxicities of these novel immune and targeted therapies, including those targeting vascular endothelial growth factor receptor (VEGFR) targets and the epidermal growth factor receptor (EGFR), will be discussed.
Keywords: chordoma; immunotherapy; chemotherapy; molecular therapies; VEGF chordoma; immunotherapy; chemotherapy; molecular therapies; VEGF

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MDPI and ACS Style

Golding, R.; Abuqubo, R.; Pansa, C.J.; Bhatta, M.; Shankar, V.; Mani, K.; Kleinbart, E.; Gelfand, Y.; Murthy, S.; De la Garza Ramos, R.; et al. Immunologic and Targeted Molecular Therapies for Chordomas: A Narrative Review. J. Clin. Med. 2024, 13, 5679. https://doi.org/10.3390/jcm13195679

AMA Style

Golding R, Abuqubo R, Pansa CJ, Bhatta M, Shankar V, Mani K, Kleinbart E, Gelfand Y, Murthy S, De la Garza Ramos R, et al. Immunologic and Targeted Molecular Therapies for Chordomas: A Narrative Review. Journal of Clinical Medicine. 2024; 13(19):5679. https://doi.org/10.3390/jcm13195679

Chicago/Turabian Style

Golding, Regina, Rami Abuqubo, Christopher J. Pansa, Manish Bhatta, Vishal Shankar, Kyle Mani, Emily Kleinbart, Yaroslav Gelfand, Saikiran Murthy, Rafael De la Garza Ramos, and et al. 2024. "Immunologic and Targeted Molecular Therapies for Chordomas: A Narrative Review" Journal of Clinical Medicine 13, no. 19: 5679. https://doi.org/10.3390/jcm13195679

APA Style

Golding, R., Abuqubo, R., Pansa, C. J., Bhatta, M., Shankar, V., Mani, K., Kleinbart, E., Gelfand, Y., Murthy, S., De la Garza Ramos, R., Krystal, J., Eleswarapu, A., Yassari, R., Mostafa, E., Fourman, M. S., & Schlumprecht, A. (2024). Immunologic and Targeted Molecular Therapies for Chordomas: A Narrative Review. Journal of Clinical Medicine, 13(19), 5679. https://doi.org/10.3390/jcm13195679

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