Chordoma—Current Understanding and Modern Treatment Paradigms
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 cavi...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-03-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/10/5/1054 |
| _version_ | 1797414732338036736 |
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| author | Sean M. Barber Saeed S. Sadrameli Jonathan J. Lee Jared S. Fridley Bin S. Teh Adetokunbo A. Oyelese Albert E. Telfeian Ziya L. Gokaslan |
| author_facet | Sean M. Barber Saeed S. Sadrameli Jonathan J. Lee Jared S. Fridley Bin S. Teh Adetokunbo A. Oyelese Albert E. Telfeian Ziya L. Gokaslan |
| author_sort | Sean M. Barber |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-09T05:37:50Z |
| format | Article |
| id | doaj.art-87c7b04ce6044cadac2a7c87c6562594 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T05:37:50Z |
| publishDate | 2021-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-87c7b04ce6044cadac2a7c87c65625942023-12-03T12:27:34ZengMDPI AGJournal of Clinical Medicine2077-03832021-03-01105105410.3390/jcm10051054Chordoma—Current Understanding and Modern Treatment ParadigmsSean M. Barber0Saeed S. Sadrameli1Jonathan J. Lee2Jared S. Fridley3Bin S. Teh4Adetokunbo A. Oyelese5Albert E. Telfeian6Ziya L. Gokaslan7Department of Neurosurgery, Houston Methodist Neurological Institute, Houston Methodist Hospital, Houston, TX 77030, USADepartment of Neurosurgery, Houston Methodist Neurological Institute, Houston Methodist Hospital, Houston, TX 77030, USADepartment of Neurosurgery, Houston Methodist Neurological Institute, Houston Methodist Hospital, Houston, TX 77030, USADepartment of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical School at Brown University, Providence, RI 02903, USADepartment of Radiation Oncology, Houston Methodist Neurological Institute, Houston Methodist Hospital, Houston, TX 77030, USADepartment of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical School at Brown University, Providence, RI 02903, USADepartment of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical School at Brown University, Providence, RI 02903, USADepartment of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical School at Brown University, Providence, RI 02903, USAChordoma 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.https://www.mdpi.com/2077-0383/10/5/1054chordomaspinal oncologyspinal tumor |
| spellingShingle | Sean M. Barber Saeed S. Sadrameli Jonathan J. Lee Jared S. Fridley Bin S. Teh Adetokunbo A. Oyelese Albert E. Telfeian Ziya L. Gokaslan Chordoma—Current Understanding and Modern Treatment Paradigms Journal of Clinical Medicine chordoma spinal oncology spinal tumor |
| title | Chordoma—Current Understanding and Modern Treatment Paradigms |
| title_full | Chordoma—Current Understanding and Modern Treatment Paradigms |
| title_fullStr | Chordoma—Current Understanding and Modern Treatment Paradigms |
| title_full_unstemmed | Chordoma—Current Understanding and Modern Treatment Paradigms |
| title_short | Chordoma—Current Understanding and Modern Treatment Paradigms |
| title_sort | chordoma current understanding and modern treatment paradigms |
| topic | chordoma spinal oncology spinal tumor |
| url | https://www.mdpi.com/2077-0383/10/5/1054 |
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