2021
DOI: 10.1016/j.jhepr.2020.100174
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New insights into the pathophysiology and clinical care of rare primary liver cancers

Abstract: 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 th… Show more

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Cited by 30 publications
(46 citation statements)
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References 157 publications
(241 reference statements)
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“…Pathologic diagnosis of cHCC‐CCA was defined as a tumour containing unequivocal elements of both hepatocytic and cholangiocytic differentiation 1,16,17 . The case of separate HCC and iCCA arising in the same liver was excluded 1,16‐18 . The biphenotypic differentiation is based on the microscopic features of haematoxylin‐eosin‐stained sections with the assistance of immunohistochemical staining results, such as hepatocyte paraffin‐1, alpha‐fetoprotein, polyclonal carcinoembryonic antigen, CK7, CK19 and/or CD10 2 .…”
Section: Methodsmentioning
confidence: 99%
See 1 more Smart Citation
“…Pathologic diagnosis of cHCC‐CCA was defined as a tumour containing unequivocal elements of both hepatocytic and cholangiocytic differentiation 1,16,17 . The case of separate HCC and iCCA arising in the same liver was excluded 1,16‐18 . The biphenotypic differentiation is based on the microscopic features of haematoxylin‐eosin‐stained sections with the assistance of immunohistochemical staining results, such as hepatocyte paraffin‐1, alpha‐fetoprotein, polyclonal carcinoembryonic antigen, CK7, CK19 and/or CD10 2 .…”
Section: Methodsmentioning
confidence: 99%
“…Contrast‐enhanced CT or MRI examinations were performed at 3‐ to 6‐month intervals after surgery. If extrahepatic recurrence was suspected based on clinical symptoms or increasing tumour marker levels, patients underwent chest CT and 18 F‐fluorodeoxyglucose positron emission tomography scanning.…”
Section: Methodsmentioning
confidence: 99%
“…Recent evidence suggest that cHCC‐CCA have a monoclonal origin. Although some have alterations similar to ICC, molecular alterations are more frequently similar to those found in HCC 10,11 . Notably, the frequency of targetable fibroblast growth factor receptor (FGFR) fusions and isocitrate dehydrogenase (IDH)1 mutations are less frequent in combined tumours, but are still found in some patients, suggesting that a systematic approach to identifying alterations may be important for the future 12 …”
Section: Figurementioning
confidence: 99%
“…Although some have alterations similar to ICC, molecular alterations are more frequently similar to those found in HCC. 10,11 Notably, the frequency of targetable fibroblast growth factor receptor (FGFR) fusions and isocitrate dehydrogenase (IDH)1 mutations are less frequent in combined tumours, but are still found in some patients, suggesting that a systematic approach to identifying alterations may be important for the future. 12 In summary, although a consensus on the pathological definition and classification of cHCC-CCA has recently been reached, there is no similar consensus regarding optimal systemic therapy for patients with advanced disease.…”
mentioning
confidence: 99%
“…The study clearly indicates that, although the registration of all RCTs before enrollment of participants is mandatory for publication by the International Committee of Medical Journal Editors, registration should be reinforced because it is key to transparency in trial reporting. Finally, in a review focusing on rare primary liver cancers (PLCs), Gigante et al 5 provide an update on the management of hepatocholangiocarcinoma, fibrolamellar carcinoma, hepatic hemangioendothelioma and hepatic sarcoma. The authors describe several advances in terms of pathophysiology, genetic profile and diagnosis but also discuss the limited data on PLC treatment.…”
mentioning
confidence: 99%