Molecular alterations and therapeutic targets in pancreatic neuroendocrine tumors
Human pancreatic neuroendocrine tumors (PanNETs) are a rare, deadly tumor type that is sporadic or arises in the background of a hereditary syndrome. A critical genetic event in sporadic tumors is inactivation of the gene menin 1 (MEN1) on chromosome 11, and indeed, PanNETs occur in patients with th...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Health/LWW
2023-09-01
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| Series: | Journal of Pancreatology |
| Online Access: | http://journals.lww.com/10.1097/JP9.0000000000000125 |
| _version_ | 1797671824894459904 |
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| author | Yarui Ma Xiaoyue Wang Hong Zhao Yuchen Jiao |
| author_facet | Yarui Ma Xiaoyue Wang Hong Zhao Yuchen Jiao |
| author_sort | Yarui Ma |
| collection | DOAJ |
| description | Human pancreatic neuroendocrine tumors (PanNETs) are a rare, deadly tumor type that is sporadic or arises in the background of a hereditary syndrome. A critical genetic event in sporadic tumors is inactivation of the gene menin 1 (MEN1) on chromosome 11, and indeed, PanNETs occur in patients with the hereditary syndrome multiple endocrine neoplasia type 1 (MEN1) due to germline mutations in the gene. Here, we review the recent progress in the field of molecular genetics and therapeutic targets of PanNETs. The key genomic alterations, including MEN1, ATRX/DAXX, mammalian target of rapamycin (mTOR), DNA damage and repair associated genes, vascular endothelial growth factor receptor (VEGFR) and SSTRs, and epigenetic aberrations in PanNETs are discussed. In addition, the commonly used preclinical models for PanNETs are enumerated. |
| first_indexed | 2024-03-11T21:21:07Z |
| format | Article |
| id | doaj.art-9f5c65dd4e25456291a46078bf781805 |
| institution | Directory Open Access Journal |
| issn | 2096-5664 |
| language | English |
| last_indexed | 2024-03-11T21:21:07Z |
| publishDate | 2023-09-01 |
| publisher | Wolters Kluwer Health/LWW |
| record_format | Article |
| series | Journal of Pancreatology |
| spelling | doaj.art-9f5c65dd4e25456291a46078bf7818052023-09-28T07:10:52ZengWolters Kluwer Health/LWWJournal of Pancreatology2096-56642023-09-016311912610.1097/JP9.0000000000000125202309000-00005Molecular alterations and therapeutic targets in pancreatic neuroendocrine tumorsYarui Ma0Xiaoyue Wang1Hong Zhao2Yuchen Jiao3a State Key Laboratory of Molecular Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, Chinab Center for Bioinformatics, School of Basic Medicine Peking Union Medical College, Institute of Basic Medical Sciences Chinese Academy of Medical Sciences, Beijing, Chinac Department of Hepatobiliary Surgery, State Key Laboratory of Molecular Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, Chinaa State Key Laboratory of Molecular Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, ChinaHuman pancreatic neuroendocrine tumors (PanNETs) are a rare, deadly tumor type that is sporadic or arises in the background of a hereditary syndrome. A critical genetic event in sporadic tumors is inactivation of the gene menin 1 (MEN1) on chromosome 11, and indeed, PanNETs occur in patients with the hereditary syndrome multiple endocrine neoplasia type 1 (MEN1) due to germline mutations in the gene. Here, we review the recent progress in the field of molecular genetics and therapeutic targets of PanNETs. The key genomic alterations, including MEN1, ATRX/DAXX, mammalian target of rapamycin (mTOR), DNA damage and repair associated genes, vascular endothelial growth factor receptor (VEGFR) and SSTRs, and epigenetic aberrations in PanNETs are discussed. In addition, the commonly used preclinical models for PanNETs are enumerated.http://journals.lww.com/10.1097/JP9.0000000000000125 |
| spellingShingle | Yarui Ma Xiaoyue Wang Hong Zhao Yuchen Jiao Molecular alterations and therapeutic targets in pancreatic neuroendocrine tumors Journal of Pancreatology |
| title | Molecular alterations and therapeutic targets in pancreatic neuroendocrine tumors |
| title_full | Molecular alterations and therapeutic targets in pancreatic neuroendocrine tumors |
| title_fullStr | Molecular alterations and therapeutic targets in pancreatic neuroendocrine tumors |
| title_full_unstemmed | Molecular alterations and therapeutic targets in pancreatic neuroendocrine tumors |
| title_short | Molecular alterations and therapeutic targets in pancreatic neuroendocrine tumors |
| title_sort | molecular alterations and therapeutic targets in pancreatic neuroendocrine tumors |
| url | http://journals.lww.com/10.1097/JP9.0000000000000125 |
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