Emerging Therapeutic Targets in Chordomas: A Review of the Literature in the Genomic Era.
Neurosurgery
; 86(2): E118-E123, 2020 02 01.
Article
in En
| MEDLINE
| ID: mdl-31504814
ABSTRACT
Chordomas are rare primary malignant tumors of the bones that occur along the skull base, spine, and sacrum. Long-term survival and neurological outcome continue to be challenging with continued low percentages of long-term survival. Recent studies have used genome, exome, transcriptome, and proteome sequencing to assess the mutational profile of chordomas. Most notably, Brachyury, or T-protein, has been shown to be an early mutational event in chordoma evolution. Clinically actionable mutations, including in the PI3K pathway, were identified. Preliminary evidence suggests that there may be mutational differences associated with primary tumor location. In this study, we review the therapeutic landscape of chordomas and discuss emerging targets in the genomic era.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Spinal Neoplasms
/
Genetic Variation
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Chordoma
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Skull Base Neoplasms
/
Genomics
Type of study:
Diagnostic_studies
/
Prognostic_studies
Limits:
Humans
Language:
En
Journal:
Neurosurgery
Year:
2020
Document type:
Article