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Epidemiology, clinical manifestations, diagnosis, and treatment of fibrolamellar carcinoma

Authors
Richard S Kalman, MD
Ghassan K Abou-Alfa, MD
Section Editors
Richard M Goldberg, MD
Alberto S Pappo, MD
Kenneth K Tanabe, MD
Deputy Editor
Diane MF Savarese, MD

INTRODUCTION

Primary malignant liver tumors resemble and arise from the major constituent cells of the liver, namely hepatocytes (giving rise to hepatocellular carcinoma [HCC]), biliary epithelial cells (cholangiocarcinoma and biliary cystadenocarcinoma), endothelial cells (angiosarcoma, epithelioid hemangioendothelioma), or combinations of these cells with various mesenchymal cells (eg, hepatoblastoma) [1]. Some (eg, combined hepatocellular-cholangiocellular carcinoma) represent collision of two different tumors or may result from malignant transformation of hepatic progenitor cells with differentiation along two different cell lineages.

HCC is the most common primary liver tumor, followed by cholangiocarcinoma. (See "Epidemiology and etiologic associations of hepatocellular carcinoma" and "Epidemiology, pathogenesis, and classification of cholangiocarcinoma".)  

This topic review will cover the epidemiology, clinical manifestations, diagnosis, and treatment of primary liver tumors other than HCC and cholangiocarcinoma. Pathology of all primary malignant liver tumors and their precursors is discussed elsewhere. (See "Pathology of malignant liver tumors".)

EPIDEMIOLOGY

Fibrolamellar carcinoma (FLC) was historically thought to be a variant of primary hepatocellular carcinoma (HCC) [2]. It was originally called eosinophilic hepatoma with lamellar fibrosis in view of its distinguishing features: tumor cells with eosinophilic cytoplasm and parallel arrangement of the collagen in conspicuous fibrous septa. (See "Pathology of malignant liver tumors", section on 'Fibrolamellar carcinoma'.)

FLC is a very rare tumor, although the incidence varies geographically. In the United States and Thailand, less than 1 percent of all primary liver tumors are FLC [3,4], while in Mexico, FLC represents 5.8 percent of all primary liver cancers [4,5].

                      
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Literature review current through: Sep 2017. | This topic last updated: Oct 10, 2017.
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