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New insights into the pathophysiology and clinical care of rare primary liver cancers
- Source :
- JHEP Reports Innovation in Hepatology, JHEP Reports Innovation in Hepatology, Elsevier, 2021, 3, pp.100174-. ⟨10.1016/j.jhepr.2020.100174⟩, JHEP Reports, JHEP Reports Innovation in Hepatology, 2021, 3, pp.100174-. ⟨10.1016/j.jhepr.2020.100174⟩
- Publication Year :
- 2021
- Publisher :
- HAL CCSD, 2021.
-
Abstract
- International audience; 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.
- Subjects :
- Pathology
Hepatocellular carcinoma
medicine.medical_treatment
liver imaging reporting and data system
Review
Liver transplantation
selective internal radiation therapy
WHO
0302 clinical medicine
SIRT
HCC
cHCC-CCA
fluorescence in situ hybridisation
AFP, alpha-fetoprotein
HAS, hepatic angiosarcoma
Selective internal radiation therapy
Gastroenterology
Epithelial cell adhesion molecule
Mixed tumor
hepatic epithelioid haemangioendothelioma
3. Good health
[SDV] Life Sciences [q-bio]
EpCAM, epithelial cell adhesion molecule
HEH, hepatic epithelioid haemangioendothelioma
030220 oncology & carcinogenesis
LI-RADS
030211 gastroenterology & hepatology
cHCC-CCA, combined hepatocholangiocarcinoma
Fibrolamellar Carcinoma
IHC, immunohistochemistry
CK, cytokeratin
medicine.medical_specialty
FLC, fibrolamellar carcinoma
CCA, cholangiocarcinoma
APHE
AFP
CEUS, contrast-enhanced ultrasound
RT-PCR
Hepatic hemangioendothelioma
LI-RADS, liver imaging reporting and data system
World Health Organization
LT, liver transplantation
WHO, World Health Organization
03 medical and health sciences
FISH
CA19-9, carbohydrate antigen 19-9
5-FU
CCA
TACE
arterial phase hyperenhancement
HEH
HepPar1
RT-PCR, reverse transcription PCR
Fibrolamellar carcinoma
medicine.disease
chemistry
CLC
EpCAM
CEUS
CLC, cholangiolocellular carcinoma
cytokeratin
Hepatocholangiocarcinoma
IHC
[SDV]Life Sciences [q-bio]
combined hepatocholangiocarcinoma
Hepatic angiosarcoma
chemistry.chemical_compound
reverse transcription PCR
LT
intrahepatic cholangiocarcinoma
5-FU, 5-Fluorouracil
Immunology and Allergy
iCCA, intrahepatic cholangiocarcinoma
Intrahepatic Cholangiocarcinoma
liver transplantation
CA19-9
carbohydrate antigen 19-9
FISH, fluorescence in situ hybridisation
FLC
SIRT, selective internal radiation therapy
CK
immunohistochemistry
cholangiolocellular carcinoma
cholangiocarcinoma
epithelial cell adhesion molecule
5-Fluorouracil
alpha-fetoprotein
Internal Medicine
medicine
HepPar1, hepatocyte specific antigen antibody
transarterial chemoembolisation
hepatocyte specific antigen antibody
iCCA
Hepatology
business.industry
TACE, transarterial chemoembolisation
HAS
APHE, arterial phase hyperenhancement
business
HCC, hepatocellular carcinoma
contrast-enhanced ultrasound
Subjects
Details
- Language :
- English
- ISSN :
- 25895559
- Database :
- OpenAIRE
- Journal :
- JHEP Reports Innovation in Hepatology, JHEP Reports Innovation in Hepatology, Elsevier, 2021, 3, pp.100174-. ⟨10.1016/j.jhepr.2020.100174⟩, JHEP Reports, JHEP Reports Innovation in Hepatology, 2021, 3, pp.100174-. ⟨10.1016/j.jhepr.2020.100174⟩
- Accession number :
- edsair.doi.dedup.....b4ab7088568fdb8caf56f1efb63cf2cd