Biphenotypic (hepatobiliary) primary liver carcinomas [B(H-B)PLCs] are rare tumors representing a heterogenous group of primary liver malignancies with evidence of both biliary and hepatocellular differentiation. These tumors have been referred to by several other names, including hepatocholangiocellular carcinoma, mixed hepatocellular cholangiocarcinoma, and combined hepatocellular cholangiocarcinoma. These tumors account for approximately 1% to 14% of all primary liver cancers.1 B(H-B)PLCs have been increasingly recognized as distinct from hepatocellular carcinomas (HCCs) and intrahepatic cholangiocarcinomas (ICCs) since the first report in 1903 by Wells2 and further characterization by Allen and Lisa3 in 1949. In general, patients with B(H-B)PLCs tend to have a worse prognosis than those with HCCs,4,5 with overall survival (OS) outcomes similar to those of ICCs; however, this is controversial, because studies have shown variability in outcomes, likely due to small sample sizes.6,7 No clear guidelines exist regarding the management of B(H-B)PLCs. Hepatic resection remains the preferred treatment; however, for patients with unresectable lesions, locoregional or systemic therapy is considered. HCCs and ICCs respond poorly to chemotherapy, and novel targeted agents are currently being explored in the treatment of these tumors.
This article reports a case of a patient with metastatic B(H-B)PLC who experienced a complete response on imaging after treatment with the combination of an epidermal growth factor receptor (EGFR) inhibitor and a vascular endothelial growth factor (VEGF) inhibitor.
Drs. Zhou, Amin, Fowler, and Keller have no financial interests, arrangements, affiliations, or commercial interests with the manufacturers of any products discussed in this article or their competitors. Dr. Brunt has disclosed that she is the co-moderator of the International Pathology Consensus Consortium. Dr. Tan receives grant/research support from Genentech.
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