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Systematic Review ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Oncol. | doi: 10.3389/fonc.2019.00030

Molecular Targeted Therapy in the Treatment of Chordoma: a Systematic Review

  • 1Shanghai Tongji Hospital, Tongji University School of Medicine, China
  • 2Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, China
  • 3School of Life Sciences and Technology, Shanghai Tenth People's Hospital, Tongji University, China
  • 4Beth Israel Deaconess Medical Center, Harvard Medical School, United States

Objectives: Chordoma is a rare bone malignancy that affects the spine and skull base. Treatment dilemma leads to a high rate of local relapse and distant metastases. Molecular targeted therapy (MTT) is an option for advanced chordoma, but its therapeutic efficacy and safety have not been investigated systematically. Therefore, a systematic review was conducted on studies reporting MTT regimens for chordoma.
Methods: Clinical trials, case series and case reports on chordoma MTT were identified using MEDLINE, Cochrane library and EMBASE, and systematically reviewed. Data on clinical outcomes such as median overall survival, progression-free survival, response rate and adverse events (AEs) were extracted and analyzed.
Results: Thirty-three eligible studies were selected for the systematic review, which indicated that imatinib and erlotinib were the most frequently used molecular targeted inhibitors (MTIs) for chordoma. For PDGFR-positive and/or EGFR-positive chordoma, clinical benefits were achieved with acceptable AEs. Monotherapy is preferred as the first-line of treatment, and combined drug therapy as the second-line treatment. In addition, the brachyury vaccine has shown promising results.
Conclusions: The selection of MTIs for patients with advanced or relapsed chordoma should be based on gene mutation screening and immunohistochemistry (IHC). Monotherapy of TKIs is recommended as the first-line management, and combination therapy (two TKIs or TKI plus mTOR inhibitor) may be the choice for drug-resistant chordoma. Brachyury vaccine is a promising therapeutic strategy and requires more clinical trials to evaluate its safety and efficacy.

Keywords: Molecular Targeted Therapy, Chordoma, Systematic review, Imatinib, erlotinib, bone tumor

Received: 05 Nov 2018; Accepted: 10 Jan 2019.

Edited by:

Jian-ye Zhang, Guangzhou Medical University, China

Reviewed by:

Claudia Palena, National Cancer Institute (NCI), United States
Victor C. Kok, Asia University, Taiwan  

Copyright: © 2019 Meng, Yin, Jin, Jiang, Huang, Song, Wang and Cheng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence:
Dr. Dianwen Song, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200080, China,
Dr. Liming Cheng, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai, China,