Epidemiology, clinical manifestations, diagnosis, and treatment of fibrolamellar carcinoma
- Richard S Kalman, MD
Richard S Kalman, MD
- Division of Hepatology,
- Assistant Professor of Medicine,
- Einstein Medical Center/Sidney Kimmel Medical College of Thomas Jefferson University
- Ghassan K Abou-Alfa, MD
Ghassan K Abou-Alfa, MD
- Associate Attending
- Memorial Sloan Kettering Cancer Center
- Associate Professor
- Weill Cornell Medi
- Section Editors
- Richard M Goldberg, MD
Richard M Goldberg, MD
- Section Editor — Gastrointestinal Cancer
- Director of the West Virginia University Cancer Institute and the Mary Babb Randolph Cancer Center
- Professor of Medicine
- Laurence S. & Jean J. DeLynn Chair of Oncology
- Alberto S Pappo, MD
Alberto S Pappo, MD
- Section Editor — Pediatric Oncology
- Head of Solid Malignancies Program
- St. Jude Children's Research Hospital
- Kenneth K Tanabe, MD
Kenneth K Tanabe, MD
- Section Editor — Gastrointestinal Malignancies
- Professor of Surgery
- Harvard Medical School
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) . 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".)
Fibrolamellar carcinoma (FLC) was historically thought to be a variant of primary hepatocellular carcinoma (HCC) . 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].To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- ETIOLOGY AND PATHOGENESIS
- CLINICAL PRESENTATION
- Tumor markers
- - Molecular diagnostics
- Differential diagnosis
- PROGNOSIS AND PROGNOSTIC FACTORS
- Potentially resectable disease
- - Posttreatment surveillance
- Unresectable cases
- - Liver transplantation
- - Systemic therapy
- Cytotoxic chemotherapy
- Targeted agents
- Clinical trials
- - Embolization
- - Radiation therapy
- SUMMARY AND RECOMMENDATIONS