New prospects for molecular targets for chordomas Review


Authors: Ozair, M. Z.; Pinkesh Shah, P.; Mathios, D.; Lim, M.; Moss, N. S.
Review Title: New prospects for molecular targets for chordomas
Abstract: Chordomas are benign, highly recurrent tumors of the midline skeleton that arise from the remnants of the notochord. The development of systemic therapy is critically important to ultimately managing this tumor. Several ongoing trials are attempting to use molecular targeted therapies for mutated pathways in recurrent and advanced chordomas and have shown promise. In addition, immunotherapies, including brachyury-directed vaccination and checkpoint inhibition, have also been attempted with encouraging results. This article discusses the major pathways that have been implicated in the pathogenesis of chordoma with an emphasis on molecular vulnerabilities that future therapies are attempting to exploit. © 2019 Elsevier Inc.
Keywords: unclassified drug; gene mutation; overall survival; histopathology; review; sorafenib; erlotinib; placebo; sunitinib; nonhuman; drug targeting; adjuvant therapy; imatinib; ticilimumab; cancer immunotherapy; progression free survival; granulocyte macrophage colony stimulating factor; transcription factor; dasatinib; protein tyrosine kinase; cetuximab; carcinogenesis; temsirolimus; gene rearrangement; immune response; immunotherapy; cancer vaccine; epigenetics; gefitinib; pazopanib; trabectedin; tumor cell vaccine; cell cycle regulation; chordoma; rapamycin; lapatinib; olaparib; tumor microenvironment; molecularly targeted therapy; notochord; molecular targeted therapy; brachyury; nivolumab; talimogene laherparepvec; human; priority journal; palbociclib; durvalumab; tazemetostat; avelumab; receptor tyrosine kinase (rtks); brachyury vaccine; gi 6301; relatlimab
Journal Title: Neurosurgery Clinics of North America
Volume: 31
Issue: 2
ISSN: 1042-3680
Publisher: W.B. Saunders Co-Elsevier Inc.  
Date Published: 2020-04-01
Start Page: 289
End Page: 300
Language: English
DOI: 10.1016/j.nec.2019.11.004
PUBMED: 32147018
PROVIDER: scopus
PMCID: PMC7374924
DOI/URL:
Notes: Review -- Export Date: 1 April 2020 -- Source: Scopus
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  1. Nelson Moss
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