In vitro and in vivo modeling systems of supratentorial ependymomas
Ependymomas are rare brain tumors that can occur in both children and adults. Subdivided by the tumors’ initial location, ependymomas develop in the central nervous system in the supratentorial or infratentorial/posterior fossa region, or the spinal cord. Supratentorial ependymomas (ST-EPNs) are pre...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2024-02-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2024.1360358/full |
| _version_ | 1827341558165274624 |
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| author | Emily A. Hatanaka Emily A. Hatanaka Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig |
| author_facet | Emily A. Hatanaka Emily A. Hatanaka Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig |
| author_sort | Emily A. Hatanaka |
| collection | DOAJ |
| description | Ependymomas are rare brain tumors that can occur in both children and adults. Subdivided by the tumors’ initial location, ependymomas develop in the central nervous system in the supratentorial or infratentorial/posterior fossa region, or the spinal cord. Supratentorial ependymomas (ST-EPNs) are predominantly characterized by common driver gene fusions such as ZFTA and YAP1 fusions. Some variants of ST-EPNs carry a high overall survival rate. In poorly responding ST-EPN variants, high levels of inter- and intratumoral heterogeneity, limited therapeutic strategies, and tumor recurrence are among the reasons for poor patient outcomes with other ST-EPN subtypes. Thus, modeling these molecular profiles is key in further studying tumorigenesis. Due to the scarcity of patient samples, the development of preclinical in vitro and in vivo models that recapitulate patient tumors is imperative when testing therapeutic approaches for this rare cancer. In this review, we will survey ST-EPN modeling systems, addressing the strengths and limitations, application for therapeutic targeting, and current literature findings. |
| first_indexed | 2024-03-07T21:42:49Z |
| format | Article |
| id | doaj.art-b7c836d1f04649dea07e86982669f055 |
| institution | Directory Open Access Journal |
| issn | 2234-943X |
| language | English |
| last_indexed | 2024-03-07T21:42:49Z |
| publishDate | 2024-02-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj.art-b7c836d1f04649dea07e86982669f0552024-02-26T04:49:16ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2024-02-011410.3389/fonc.2024.13603581360358In vitro and in vivo modeling systems of supratentorial ependymomasEmily A. Hatanaka0Emily A. Hatanaka1Joshua J. Breunig2Joshua J. Breunig3Joshua J. Breunig4Joshua J. Breunig5Joshua J. Breunig6Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United StatesDepartment of Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA, United StatesBoard of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United StatesDepartment of Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA, United StatesCenter for Neural Sciences in Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, United StatesSamuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United StatesDepartment of Medicine, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, United StatesEpendymomas are rare brain tumors that can occur in both children and adults. Subdivided by the tumors’ initial location, ependymomas develop in the central nervous system in the supratentorial or infratentorial/posterior fossa region, or the spinal cord. Supratentorial ependymomas (ST-EPNs) are predominantly characterized by common driver gene fusions such as ZFTA and YAP1 fusions. Some variants of ST-EPNs carry a high overall survival rate. In poorly responding ST-EPN variants, high levels of inter- and intratumoral heterogeneity, limited therapeutic strategies, and tumor recurrence are among the reasons for poor patient outcomes with other ST-EPN subtypes. Thus, modeling these molecular profiles is key in further studying tumorigenesis. Due to the scarcity of patient samples, the development of preclinical in vitro and in vivo models that recapitulate patient tumors is imperative when testing therapeutic approaches for this rare cancer. In this review, we will survey ST-EPN modeling systems, addressing the strengths and limitations, application for therapeutic targeting, and current literature findings.https://www.frontiersin.org/articles/10.3389/fonc.2024.1360358/fullgliomaoncofusion proteinsrare cancertumor modelingpediatric tumorbrain cancer |
| spellingShingle | Emily A. Hatanaka Emily A. Hatanaka Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig Joshua J. Breunig In vitro and in vivo modeling systems of supratentorial ependymomas Frontiers in Oncology glioma oncofusion proteins rare cancer tumor modeling pediatric tumor brain cancer |
| title | In vitro and in vivo modeling systems of supratentorial ependymomas |
| title_full | In vitro and in vivo modeling systems of supratentorial ependymomas |
| title_fullStr | In vitro and in vivo modeling systems of supratentorial ependymomas |
| title_full_unstemmed | In vitro and in vivo modeling systems of supratentorial ependymomas |
| title_short | In vitro and in vivo modeling systems of supratentorial ependymomas |
| title_sort | in vitro and in vivo modeling systems of supratentorial ependymomas |
| topic | glioma oncofusion proteins rare cancer tumor modeling pediatric tumor brain cancer |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2024.1360358/full |
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