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Fibrolamellar Carcinoma: Recent Advances and Unresolved Questions on the Molecular Mechanisms.

ABSTRACT: Fibrolamellar hepatocellular carcinoma (FLC) is a rare form of primary liver cancer that affects adolescents and young adults without underlying liver disease. Surgery remains the mainstay of therapy; however, most patients are either not surgical candidates or suffer from recurrence. There is no approved systemic therapy and the overall survival remains poor. Historically classified as a subtype of hepatocellular carcinoma (HCC), FLC has a unique clinical, histological, and molecular presentation. At the genomic level, FLC contains a single 400kB deletion in chromosome 19, leading to a functional DNAJB1-PRKACA fusion protein. In this review, we detail the recent advances in our understanding of the molecular underpinnings of FLC and outline the current knowledge gaps.

SUBMITTER: Lalazar G

PROVIDER: S-EPMC6020845 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature

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Fibrolamellar Carcinoma: Recent Advances and Unresolved Questions on the Molecular Mechanisms.

Lalazar Gadi G Simon Sanford M SM

Seminars in liver disease 20180222 1

Fibrolamellar hepatocellular carcinoma (FLC) is a rare form of primary liver cancer that affects adolescents and young adults without underlying liver disease. Surgery remains the mainstay of therapy; however, most patients are either not surgical candidates or suffer from recurrence. There is no approved systemic therapy and the overall survival remains poor. Historically classified as a subtype of hepatocellular carcinoma (HCC), FLC has a unique clinical, histological, and molecular presentati ...[more]

PMID: 29471565

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