Translational Windows in Chordoma: A Target Appraisal
Chordomas are rare tumors that are notoriously refractory to chemotherapy and radiotherapy when radical surgical resection is not achieved or upon recurrence after maximally aggressive treatment. The study of chordomas has been complicated by small patient cohorts and few available model systems due...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2020-07-01
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| Series: | Frontiers in Neurology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/article/10.3389/fneur.2020.00657/full |
| _version_ | 1819066891697651712 |
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| author | Samantha E. Hoffman Sally A. Al Abdulmohsen Saksham Gupta Blake M. Hauser David M. Meredith Ian F. Dunn Wenya Linda Bi |
| author_facet | Samantha E. Hoffman Sally A. Al Abdulmohsen Saksham Gupta Blake M. Hauser David M. Meredith Ian F. Dunn Wenya Linda Bi |
| author_sort | Samantha E. Hoffman |
| collection | DOAJ |
| description | Chordomas are rare tumors that are notoriously refractory to chemotherapy and radiotherapy when radical surgical resection is not achieved or upon recurrence after maximally aggressive treatment. The study of chordomas has been complicated by small patient cohorts and few available model systems due to the rarity of these tumors. Emerging next-generation sequencing technologies have broadened understanding of this disease by implicating novel pathways for possible targeted therapy. Mutations in cell-cycle regulation and chromatin remodeling genes have been identified in chordomas, but their significance remains unknown. Investigation of the immune microenvironment of these tumors suggests that checkpoint protein expression may influence prognosis, and adjuvant immunotherapy may improve patient outcome. Finally, growing evidence supports aberrant growth factor signaling as potential pathogenic mechanisms in chordoma. In this review, we characterize the impact on treatment opportunities offered by the genomic and immunologic landscape of this tumor. |
| first_indexed | 2024-12-21T16:09:34Z |
| format | Article |
| id | doaj.art-f3cc77af15e04ae3a6d793f961ff4934 |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-12-21T16:09:34Z |
| publishDate | 2020-07-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-f3cc77af15e04ae3a6d793f961ff49342022-12-21T18:57:49ZengFrontiers Media S.A.Frontiers in Neurology1664-22952020-07-011110.3389/fneur.2020.00657539928Translational Windows in Chordoma: A Target AppraisalSamantha E. Hoffman0Sally A. Al Abdulmohsen1Saksham Gupta2Blake M. Hauser3David M. Meredith4Ian F. Dunn5Wenya Linda Bi6Center for Skull Base and Pituitary Surgery, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United StatesCenter for Skull Base and Pituitary Surgery, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United StatesCenter for Skull Base and Pituitary Surgery, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United StatesCenter for Skull Base and Pituitary Surgery, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United StatesDepartment of Pathology, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United StatesDepartment of Neurosurgery, University of Oklahoma College of Medicine, Oklahoma City, OK, United StatesCenter for Skull Base and Pituitary Surgery, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United StatesChordomas are rare tumors that are notoriously refractory to chemotherapy and radiotherapy when radical surgical resection is not achieved or upon recurrence after maximally aggressive treatment. The study of chordomas has been complicated by small patient cohorts and few available model systems due to the rarity of these tumors. Emerging next-generation sequencing technologies have broadened understanding of this disease by implicating novel pathways for possible targeted therapy. Mutations in cell-cycle regulation and chromatin remodeling genes have been identified in chordomas, but their significance remains unknown. Investigation of the immune microenvironment of these tumors suggests that checkpoint protein expression may influence prognosis, and adjuvant immunotherapy may improve patient outcome. Finally, growing evidence supports aberrant growth factor signaling as potential pathogenic mechanisms in chordoma. In this review, we characterize the impact on treatment opportunities offered by the genomic and immunologic landscape of this tumor.https://www.frontiersin.org/article/10.3389/fneur.2020.00657/fullchordomagenomicsimmunologytargeted therapycheckpoint inhibition |
| spellingShingle | Samantha E. Hoffman Sally A. Al Abdulmohsen Saksham Gupta Blake M. Hauser David M. Meredith Ian F. Dunn Wenya Linda Bi Translational Windows in Chordoma: A Target Appraisal Frontiers in Neurology chordoma genomics immunology targeted therapy checkpoint inhibition |
| title | Translational Windows in Chordoma: A Target Appraisal |
| title_full | Translational Windows in Chordoma: A Target Appraisal |
| title_fullStr | Translational Windows in Chordoma: A Target Appraisal |
| title_full_unstemmed | Translational Windows in Chordoma: A Target Appraisal |
| title_short | Translational Windows in Chordoma: A Target Appraisal |
| title_sort | translational windows in chordoma a target appraisal |
| topic | chordoma genomics immunology targeted therapy checkpoint inhibition |
| url | https://www.frontiersin.org/article/10.3389/fneur.2020.00657/full |
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