PMID- 33806339
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20210408
IS  - 2077-0383 (Print)
IS  - 2077-0383 (Electronic)
IS  - 2077-0383 (Linking)
VI  - 10
IP  - 5
DP  - 2021 Mar 4
TI  - Chordoma-Current Understanding and Modern Treatment Paradigms.
LID - 10.3390/jcm10051054 [doi]
LID - 1054
AB  - Chordoma is a low-grade notochordal tumor of the skull base, mobile spine and 
      sacrum which behaves malignantly and confers a poor prognosis despite indolent 
      growth patterns. These tumors often present late in the disease course, tend to 
      encapsulate adjacent neurovascular anatomy, seed resection cavities, recur 
      locally and respond poorly to radiotherapy and conventional chemotherapy, all of 
      which make chordomas challenging to treat. Extent of surgical resection and 
      adequacy of surgical margins are the most important prognostic factors and thus 
      patients with chordoma should be cared for by a highly experienced, 
      multi-disciplinary surgical team in a quaternary center. Ongoing research into 
      the molecular pathophysiology of chordoma has led to the discovery of several 
      pathways that may serve as potential targets for molecular therapy, including a 
      multitude of receptor tyrosine kinases (e.g., platelet-derived growth factor 
      receptor [PDGFR], epidermal growth factor receptor [EGFR]), downstream cascades 
      (e.g., phosphoinositide 3-kinase [PI3K]/protein kinase B [Akt]/mechanistic target 
      of rapamycin [mTOR]), brachyury-a transcription factor expressed ubiquitously in 
      chordoma but not in other tissues-and the fibroblast growth factor 
      [FGF]/mitogen-activated protein kinase kinase [MEK]/extracellular 
      signal-regulated kinase [ERK] pathway. In this review article, the 
      pathophysiology, diagnosis and modern treatment paradigms of chordoma will be 
      discussed with an emphasis on the ongoing research and advances in the field that 
      may lead to improved outcomes for patients with this challenging disease.
FAU - Barber, Sean M
AU  - Barber SM
AD  - Department of Neurosurgery, Houston Methodist Neurological Institute, Houston 
      Methodist Hospital, Houston, TX 77030, USA.
FAU - Sadrameli, Saeed S
AU  - Sadrameli SS
AD  - Department of Neurosurgery, Houston Methodist Neurological Institute, Houston 
      Methodist Hospital, Houston, TX 77030, USA.
FAU - Lee, Jonathan J
AU  - Lee JJ
AD  - Department of Neurosurgery, Houston Methodist Neurological Institute, Houston 
      Methodist Hospital, Houston, TX 77030, USA.
FAU - Fridley, Jared S
AU  - Fridley JS
AD  - Department of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical 
      School at Brown University, Providence, RI 02903, USA.
FAU - Teh, Bin S
AU  - Teh BS
AD  - Department of Radiation Oncology, Houston Methodist Neurological Institute, 
      Houston Methodist Hospital, Houston, TX 77030, USA.
FAU - Oyelese, Adetokunbo A
AU  - Oyelese AA
AD  - Department of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical 
      School at Brown University, Providence, RI 02903, USA.
FAU - Telfeian, Albert E
AU  - Telfeian AE
AD  - Department of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical 
      School at Brown University, Providence, RI 02903, USA.
FAU - Gokaslan, Ziya L
AU  - Gokaslan ZL
AD  - Department of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical 
      School at Brown University, Providence, RI 02903, USA.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20210304
PL  - Switzerland
TA  - J Clin Med
JT  - Journal of clinical medicine
JID - 101606588
PMC - PMC7961966
OTO - NOTNLM
OT  - chordoma
OT  - spinal oncology
OT  - spinal tumor
COIS- The authors declare no conflict of interest.
EDAT- 2021/04/04 06:00
MHDA- 2021/04/04 06:01
PMCR- 2021/03/04
CRDT- 2021/04/03 01:23
PHST- 2021/01/14 00:00 [received]
PHST- 2021/01/29 00:00 [revised]
PHST- 2021/01/31 00:00 [accepted]
PHST- 2021/04/03 01:23 [entrez]
PHST- 2021/04/04 06:00 [pubmed]
PHST- 2021/04/04 06:01 [medline]
PHST- 2021/03/04 00:00 [pmc-release]
AID - jcm10051054 [pii]
AID - jcm-10-01054 [pii]
AID - 10.3390/jcm10051054 [doi]
PST - epublish
SO  - J Clin Med. 2021 Mar 4;10(5):1054. doi: 10.3390/jcm10051054.