Review of the clinicopathologic features of fibrolamellar carcinoma

Research output: Contribution to journalReview article

84 Citations (Scopus)

Abstract

Since its first description 50 years ago, fibrolamellar carcinomas (FLCs) have been recognized as a unique type of primary liver cancer. FLCs occur principally in children and young adults and are not associated with chronic liver disease. Their etiology is unknown. The tumor is made up of large polygonal cells containing abundant eosinophilic cytoplasm, large vesiculated nuclei, and large nucleoli, with tumor cells that are embedded in lamellar bands of fibrosis. Although rare, the most common variant of FLC shows areas of glandular type differentiation with mucin production. The uniqueness of FLC extends to their molecular findings, as they show no evidence for involvement by many of the major pathways and genes that are dysregulated in typical hepatocellular carcinoma, including alpha-fetoprotein, TP53 mutations, and beta catenin mutations. FLCs are not indolent tumors, but have an overall better prognosis than hepatocellular carcinomas of the usual sort because of the younger age at presentation and lack of cirrhosis. The most important prognostic feature is resectability. Although their morphologic appearance on routine stains is well defined, their etiology is still unknown and much of their molecular biology remains poorly described and awaits future investigation.

Original languageEnglish (US)
Pages (from-to)217-223
Number of pages7
JournalAdvances in anatomic pathology
Volume14
Issue number3
DOIs
StatePublished - May 1 2007
Externally publishedYes

Fingerprint

Carcinoma
Hepatocellular Carcinoma
Fibrosis
Neoplasms
Mutation
alpha-Fetoproteins
beta Catenin
Mucins
Liver Neoplasms
Liver Diseases
Molecular Biology
Young Adult
Cytoplasm
Chronic Disease
Coloring Agents
Genes

Keywords

  • Fibrolamellar carcinoma
  • Hepatocellular carcinoma
  • Prognosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Anatomy

Cite this

Review of the clinicopathologic features of fibrolamellar carcinoma. / Torbenson, Michael.

In: Advances in anatomic pathology, Vol. 14, No. 3, 01.05.2007, p. 217-223.

Research output: Contribution to journalReview article

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abstract = "Since its first description 50 years ago, fibrolamellar carcinomas (FLCs) have been recognized as a unique type of primary liver cancer. FLCs occur principally in children and young adults and are not associated with chronic liver disease. Their etiology is unknown. The tumor is made up of large polygonal cells containing abundant eosinophilic cytoplasm, large vesiculated nuclei, and large nucleoli, with tumor cells that are embedded in lamellar bands of fibrosis. Although rare, the most common variant of FLC shows areas of glandular type differentiation with mucin production. The uniqueness of FLC extends to their molecular findings, as they show no evidence for involvement by many of the major pathways and genes that are dysregulated in typical hepatocellular carcinoma, including alpha-fetoprotein, TP53 mutations, and beta catenin mutations. FLCs are not indolent tumors, but have an overall better prognosis than hepatocellular carcinomas of the usual sort because of the younger age at presentation and lack of cirrhosis. The most important prognostic feature is resectability. Although their morphologic appearance on routine stains is well defined, their etiology is still unknown and much of their molecular biology remains poorly described and awaits future investigation.",
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