New insights into the pathophysiology and clinical care of rare primary liver cancers

Elia Gigante (1, 2) , Valérie Paradis (1, 3) , Maxime Ronot (1, 4) , François Cauchy (1, 3) , Olivier Soubrane (1, 3) , Nathalie Ganne-Carrié (5, 2) , Jean-Charles Nault (5, 2)

1 CRI (UMR_S_1149 / ERL_8252 / U1149) - Centre de recherche sur l'Inflammation
2 Hôpital Avicenne [AP-HP]
3 HUPNVS - Hôpitaux Universitaires Paris Nord Val de Seine
4 Hôpital Beaujon [AP-HP]
5 CRC (UMR_S_1138 / U1138) - Centre de Recherche des Cordeliers

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.

Mots clés: Hepatic hemangioendothelioma; Hepatocellular carcinoma; Hepatocholangiocarcinoma; IHC; immunohistochemistry; LI-RADS; liver imaging reporting and data system; LT; liver transplantation; Mixed tumor; RT-PCR; reverse transcription PCR; SIRT; selective internal radiation therapy; TACE; transarterial chemoembolisation; WHO; World Health Organization; cHCC-CCA; combined hepatocholangiocarcinoma; iCCA; intrahepatic cholangiocarcinoma; 5-FU; 5-Fluorouracil; AFP; alpha-fetoprotein; APHE; arterial phase hyperenhancement; CA19-9; carbohydrate antigen 19-9; CCA; cholangiocarcinoma; CEUS; contrast-enhanced ultrasound; CK; cytokeratin; CLC; cholangiolocellular carcinoma; EpCAM; epithelial cell adhesion molecule; FISH; fluorescence in situ hybridisation; FLC; fibrolamellar carcinoma; Fibrolamellar carcinoma; HAS; hepatic angiosarcoma; HCC; hepatocellular carcinoma; HEH; hepatic epithelioid haemangioendothelioma; HepPar1; hepatocyte specific antigen antibody; Hepatic angiosarcoma

Domaines: Sciences du Vivant [q-bio]

Elia Gigante, Valérie Paradis, Maxime Ronot, François Cauchy, Olivier Soubrane, et al.. New insights into the pathophysiology and clinical care of rare primary liver cancers. JHEP Reports Innovation in Hepatology, 2021, 3, pp.100174 -. ⟨10.1016/j.jhepr.2020.100174⟩. ⟨hal-03493211⟩