PMID- 30775316
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200929
IS  - 2234-943X (Print)
IS  - 2234-943X (Electronic)
IS  - 2234-943X (Linking)
VI  - 9
DP  - 2019
TI  - Molecular Targeted Therapy in the Treatment of Chordoma: A Systematic Review.
PG  - 30
LID - 10.3389/fonc.2019.00030 [doi]
LID - 30
AB  - 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.
FAU - Meng, Tong
AU  - Meng T
AD  - Division of Spine, Department of Orthopedics, Tongji Hospital Affiliated to 
      Tongji University School of Medicine, Shanghai, China.
AD  - Shanghai Bone Tumor Institution, Shanghai, China.
AD  - Department of Orthopedics, Shanghai General Hospital, School of Medicine, 
      Shanghai Jiaotong University, Shanghai, China.
FAU - Jin, Jiali
AU  - Jin J
AD  - Department of Central Laboratory, Shanghai Tenth People's Hospital of Tongji 
      University, School of Medicine, School of Life Sciences and Technology, Tongji 
      University, Shanghai, China.
FAU - Jiang, Cong
AU  - Jiang C
AD  - Beth Israel Deaconess Medical Center, BIDMC Cancer Center, Harvard Medical 
      School, Cancer Research Institute, Boston, MA, United States.
FAU - Huang, Runzhi
AU  - Huang R
AD  - Division of Spine, Department of Orthopedics, Tongji Hospital Affiliated to 
      Tongji University School of Medicine, Shanghai, China.
FAU - Yin, Huabin
AU  - Yin H
AD  - Shanghai Bone Tumor Institution, Shanghai, China.
AD  - Department of Orthopedics, Shanghai General Hospital, School of Medicine, 
      Shanghai Jiaotong University, Shanghai, China.
FAU - Song, Dianwen
AU  - Song D
AD  - Shanghai Bone Tumor Institution, Shanghai, China.
AD  - Department of Orthopedics, Shanghai General Hospital, School of Medicine, 
      Shanghai Jiaotong University, Shanghai, China.
FAU - Cheng, Liming
AU  - Cheng L
AD  - Division of Spine, Department of Orthopedics, Tongji Hospital Affiliated to 
      Tongji University School of Medicine, Shanghai, China.
AD  - Key Laboratory of Spine and Spinal Cord Injury Repair and Regeneration, Ministry 
      of Education, Tongji University, Shanghai, China.
LA  - eng
PT  - Journal Article
DEP - 20190201
PL  - Switzerland
TA  - Front Oncol
JT  - Frontiers in oncology
JID - 101568867
PMC - PMC6367227
OTO - NOTNLM
OT  - bone tumor
OT  - chordoma
OT  - erlotinib
OT  - imatinib
OT  - molecular targeted therapy
OT  - systematic review
EDAT- 2019/02/19 06:00
MHDA- 2019/02/19 06:01
PMCR- 2019/01/01
CRDT- 2019/02/19 06:00
PHST- 2018/11/05 00:00 [received]
PHST- 2019/01/10 00:00 [accepted]
PHST- 2019/02/19 06:00 [entrez]
PHST- 2019/02/19 06:00 [pubmed]
PHST- 2019/02/19 06:01 [medline]
PHST- 2019/01/01 00:00 [pmc-release]
AID - 10.3389/fonc.2019.00030 [doi]
PST - epublish
SO  - Front Oncol. 2019 Feb 1;9:30. doi: 10.3389/fonc.2019.00030. eCollection 2019.