Rare primary liver cancers: An EASL position paper

Area of Science:

• Hepatology and Oncology
• Molecular Biology
• Pathology

Background:

• Rare primary liver cancers (PLCs), such as combined hepatocellular-cholangiocarcinoma, fibrolamellar carcinoma, and hepatic epithelioid hemangioendothelioma, are gaining attention.
• Advances in understanding hepatocarcinogenesis have driven this increased focus.

Purpose of the Study:

• To summarize current information and evidence on the pathogenesis, diagnosis, and treatment of rare PLCs.
• To provide a position paper based on expert consensus.

Main Methods:

• International expert panel review.
• Synthesis of available information and evidence.
• Multidisciplinary approach including oncology, hepatology, pathology, radiology, surgery, and molecular biology.

Main Results:

• A comprehensive summary of rare PLC knowledge is presented.
• Clinical trials for systemic treatments are ongoing for certain rare PLCs.
• Current evidence indicates a need for further research.

Conclusions:

• More research, involving national and international collaboration, is required for rare PLCs.
• The multidisciplinary expert summary provides a foundation for future studies and clinical practice.