Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic Review
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 investigate...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2019-02-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fonc.2019.00030/full |
| _version_ | 1818271898691174400 |
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| author | Tong Meng Tong Meng Tong Meng Jiali Jin Cong Jiang Runzhi Huang Huabin Yin Huabin Yin Dianwen Song Dianwen Song Liming Cheng Liming Cheng |
| author_facet | Tong Meng Tong Meng Tong Meng Jiali Jin Cong Jiang Runzhi Huang Huabin Yin Huabin Yin Dianwen Song Dianwen Song Liming Cheng Liming Cheng |
| author_sort | Tong Meng |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-12T21:33:30Z |
| format | Article |
| id | doaj.art-4d36703992bc422ca7feeb3e0b6ea15f |
| institution | Directory Open Access Journal |
| issn | 2234-943X |
| language | English |
| last_indexed | 2024-12-12T21:33:30Z |
| publishDate | 2019-02-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj.art-4d36703992bc422ca7feeb3e0b6ea15f2022-12-22T00:11:14ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2019-02-01910.3389/fonc.2019.00030435088Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic ReviewTong Meng0Tong Meng1Tong Meng2Jiali Jin3Cong Jiang4Runzhi Huang5Huabin Yin6Huabin Yin7Dianwen Song8Dianwen Song9Liming Cheng10Liming Cheng11Division of Spine, Department of Orthopedics, Tongji Hospital Affiliated to Tongji University School of Medicine, Shanghai, ChinaShanghai Bone Tumor Institution, Shanghai, ChinaDepartment of Orthopedics, Shanghai General Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, ChinaDepartment of Central Laboratory, Shanghai Tenth People's Hospital of Tongji University, School of Medicine, School of Life Sciences and Technology, Tongji University, Shanghai, ChinaBeth Israel Deaconess Medical Center, BIDMC Cancer Center, Harvard Medical School, Cancer Research Institute, Boston, MA, United StatesDivision of Spine, Department of Orthopedics, Tongji Hospital Affiliated to Tongji University School of Medicine, Shanghai, ChinaShanghai Bone Tumor Institution, Shanghai, ChinaDepartment of Orthopedics, Shanghai General Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, ChinaShanghai Bone Tumor Institution, Shanghai, ChinaDepartment of Orthopedics, Shanghai General Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, ChinaDivision of Spine, Department of Orthopedics, Tongji Hospital Affiliated to Tongji University School of Medicine, Shanghai, ChinaKey Laboratory of Spine and Spinal Cord Injury Repair and Regeneration, Ministry of Education, Tongji University, Shanghai, ChinaObjectives: 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.https://www.frontiersin.org/article/10.3389/fonc.2019.00030/fullmolecular targeted therapybone tumorchordomasystematic reviewimatiniberlotinib |
| spellingShingle | Tong Meng Tong Meng Tong Meng Jiali Jin Cong Jiang Runzhi Huang Huabin Yin Huabin Yin Dianwen Song Dianwen Song Liming Cheng Liming Cheng Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic Review Frontiers in Oncology molecular targeted therapy bone tumor chordoma systematic review imatinib erlotinib |
| title | Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic Review |
| title_full | Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic Review |
| title_fullStr | Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic Review |
| title_full_unstemmed | Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic Review |
| title_short | Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic Review |
| title_sort | molecular targeted therapy in the treatment of chordoma a systematic review |
| topic | molecular targeted therapy bone tumor chordoma systematic review imatinib erlotinib |
| url | https://www.frontiersin.org/article/10.3389/fonc.2019.00030/full |
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