Targeted Therapies for Perihilar Cholangiocarcinoma
Perihilar cholangiocarcinoma (pCCA) is the anatomical sub-group of biliary tract cancer (BTC) arising between the second-order intrahepatic bile ducts and the cystic duct. Together with distal and intrahepatic cholangiocarcinoma (dCCA and iCCA; originating distal to, and proximal to this, respective...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-03-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/14/7/1789 |
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| author | Simon Gray Angela Lamarca Julien Edeline Heinz-Josef Klümpen Richard A. Hubner Mairéad G. McNamara Juan W. Valle |
| author_facet | Simon Gray Angela Lamarca Julien Edeline Heinz-Josef Klümpen Richard A. Hubner Mairéad G. McNamara Juan W. Valle |
| author_sort | Simon Gray |
| collection | DOAJ |
| description | Perihilar cholangiocarcinoma (pCCA) is the anatomical sub-group of biliary tract cancer (BTC) arising between the second-order intrahepatic bile ducts and the cystic duct. Together with distal and intrahepatic cholangiocarcinoma (dCCA and iCCA; originating distal to, and proximal to this, respectively), gallbladder cancer (GBC) and ampulla of Vater carcinoma (AVC), these clinicopathologically and molecularly distinct entities comprise biliary tract cancer (BTC). Most pCCAs are unresectable at diagnosis, and for those with resectable disease, surgery is extensive, and recurrence is common. Therefore, the majority of patients with pCCA will require systemic treatment for advanced disease. The prognosis with cytotoxic chemotherapy remains poor, driving interest in therapies targeted to the molecular nature of a given patient’s cancer. In recent years, the search for efficacious targeted therapies has been fuelled both by whole-genome and epigenomic studies, looking to uncover the molecular landscape of CCA, and by specifically testing for aberrations where established therapies exist in other indications. This review aims to provide a focus on the current molecular characterisation of pCCA, targeted therapies applicable to pCCA, and future directions in applying personalised medicine to this difficult-to-treat malignancy. |
| first_indexed | 2024-03-09T12:02:42Z |
| format | Article |
| id | doaj.art-69515cdd8cc04a1593b520d4d0d57c71 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-09T12:02:42Z |
| publishDate | 2022-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-69515cdd8cc04a1593b520d4d0d57c712023-11-30T23:02:04ZengMDPI AGCancers2072-66942022-03-01147178910.3390/cancers14071789Targeted Therapies for Perihilar CholangiocarcinomaSimon Gray0Angela Lamarca1Julien Edeline2Heinz-Josef Klümpen3Richard A. Hubner4Mairéad G. McNamara5Juan W. Valle6Department of Medical Oncology, The Christie NHS Foundation Trust, Wilmslow Rd, Manchester M20 4BX, UKDepartment of Medical Oncology, The Christie NHS Foundation Trust, Wilmslow Rd, Manchester M20 4BX, UKCentre Eugène Marquis, Av. de la Bataille Flandres Dunkerque-CS 44229, CEDEX, 35042 Rennes, FranceDepartment of Medical Oncology, Amsterdam University Medical Center, P.O. Box 7057, 1081 HV Amsterdam, The NetherlandsDepartment of Medical Oncology, The Christie NHS Foundation Trust, Wilmslow Rd, Manchester M20 4BX, UKDepartment of Medical Oncology, The Christie NHS Foundation Trust, Wilmslow Rd, Manchester M20 4BX, UKDepartment of Medical Oncology, The Christie NHS Foundation Trust, Wilmslow Rd, Manchester M20 4BX, UKPerihilar cholangiocarcinoma (pCCA) is the anatomical sub-group of biliary tract cancer (BTC) arising between the second-order intrahepatic bile ducts and the cystic duct. Together with distal and intrahepatic cholangiocarcinoma (dCCA and iCCA; originating distal to, and proximal to this, respectively), gallbladder cancer (GBC) and ampulla of Vater carcinoma (AVC), these clinicopathologically and molecularly distinct entities comprise biliary tract cancer (BTC). Most pCCAs are unresectable at diagnosis, and for those with resectable disease, surgery is extensive, and recurrence is common. Therefore, the majority of patients with pCCA will require systemic treatment for advanced disease. The prognosis with cytotoxic chemotherapy remains poor, driving interest in therapies targeted to the molecular nature of a given patient’s cancer. In recent years, the search for efficacious targeted therapies has been fuelled both by whole-genome and epigenomic studies, looking to uncover the molecular landscape of CCA, and by specifically testing for aberrations where established therapies exist in other indications. This review aims to provide a focus on the current molecular characterisation of pCCA, targeted therapies applicable to pCCA, and future directions in applying personalised medicine to this difficult-to-treat malignancy.https://www.mdpi.com/2072-6694/14/7/1789perihilarcholangiocarcinomapCCAextrahepatictargeted therapybiliary tract cancer |
| spellingShingle | Simon Gray Angela Lamarca Julien Edeline Heinz-Josef Klümpen Richard A. Hubner Mairéad G. McNamara Juan W. Valle Targeted Therapies for Perihilar Cholangiocarcinoma Cancers perihilar cholangiocarcinoma pCCA extrahepatic targeted therapy biliary tract cancer |
| title | Targeted Therapies for Perihilar Cholangiocarcinoma |
| title_full | Targeted Therapies for Perihilar Cholangiocarcinoma |
| title_fullStr | Targeted Therapies for Perihilar Cholangiocarcinoma |
| title_full_unstemmed | Targeted Therapies for Perihilar Cholangiocarcinoma |
| title_short | Targeted Therapies for Perihilar Cholangiocarcinoma |
| title_sort | targeted therapies for perihilar cholangiocarcinoma |
| topic | perihilar cholangiocarcinoma pCCA extrahepatic targeted therapy biliary tract cancer |
| url | https://www.mdpi.com/2072-6694/14/7/1789 |
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