Abstract
SummaryHepatocholangiocarcinoma, 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.
Highlights
Hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) account, respectively, for 85% and 10% of all primary liver cancers (PLCs)
Prospective studies and clinical trials are lacking for rare PLCs, such as combined hepatocholangiocarcinoma, fibrolamellar carcinoma (FLC), hepatic epithelioid haemangioendothelioma (HEH) and hepatic angiosarcoma (HAS) due to their scarcity
Combined hepatocholangiocarcinoma A matter of definition cHCC-CCA is characterised at histology by the presence of 2 distinct morphological patterns in the same lesion: HCC and intrahepatic CCA.[1,2]
Summary
Hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) account, respectively, for 85% and 10% of all primary liver cancers (PLCs). PLCs completely composed of “intermediate cells” (intermediate cell carcinoma) – small cells of intermediate size (between that of stem cells and hepatocytes) with transitional morphology between hepatocytes and cholangiocytes Whether these cells can be considered a subtype of cHCC-CCA is still a matter of discussion.[5,6]. PLCs composed of pure CLC; if the main component of PLC is >80% CLC, they can be reclassified as small duct iCCAs.[6] According to this classification, the concept of “stem cell” phenotypes based on immunohistochemistry (epithelial cell adhesion molecule [EpCAM], cytokeratin (CK)[19] and CD56) is not considered as a sub-category per se, but rather as a feature that can be present in different types of PLC.[5] all subtypes of PLC could be associated, in the same lesion, with minor histological.
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