Bibliographic Details
| Title: |
New insights into the pathophysiology and clinical care of rare primary liver cancersKey points |
| Authors: |
Elia Gigante, Valérie Paradis, Maxime Ronot, François Cauchy, Olivier Soubrane, Nathalie Ganne-Carrié, Jean-Charles Nault |
| Source: |
JHEP Reports, Vol 3, Iss 1, Pp 100174- (2021) |
| Publisher Information: |
Elsevier, 2021. |
| Publication Year: |
2021 |
| Collection: |
LCC:Diseases of the digestive system. Gastroenterology |
| Subject Terms: |
Mixed tumor, Hepatocholangiocarcinoma, Fibrolamellar carcinoma, Hepatic hemangioendothelioma, Hepatocellular carcinoma, Hepatic angiosarcoma, Diseases of the digestive system. Gastroenterology, RC799-869 |
| More Details: |
Summary: Hepatocholangiocarcinoma, fibrolamellar carcinoma, hepatic haemangioendothelioma and hepatic angiosarcoma represent less than 5% of primary liver cancers. Fibrolamellar carcinoma and hepatic haemangioendothelioma are driven by unique somatic genetic alterations (DNAJB1-PRKCA and CAMTA1-WWTR1 fusions, respectively), while the pathogenesis of hepatocholangiocarcinoma remains more complex, as suggested by its histological diversity. Histology is the gold standard for diagnosis, which remains challenging even in an expert centre because of the low incidences of these liver cancers. Resection, when feasible, is the cornerstone of treatment, together with liver transplantation for hepatic haemangioendothelioma. The role of locoregional therapies and systemic treatments remains poorly studied. In this review, we aim to describe the recent advances in terms of diagnosis and clinical management of these rare primary liver cancers. |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
2589-5559 |
| Relation: |
http://www.sciencedirect.com/science/article/pii/S2589555920301087; https://doaj.org/toc/2589-5559 |
| DOI: |
10.1016/j.jhepr.2020.100174 |
| Access URL: |
https://doaj.org/article/2925d692d0bd456dbc52d77252245475 |
| Accession Number: |
edsdoj.2925d692d0bd456dbc52d77252245475 |
| Database: |
Directory of Open Access Journals |
