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Fibrolamellar Carcinoma Clinical Presentation

  • Author: Michael A Choti, MD, MBA, FACS; Chief Editor: N Joseph Espat, MD, MS, FACS  more...
 
Updated: Sep 17, 2015
 

History

Fibrolamellar carcinoma (FLC) typically presents with nonspecific symptoms or no symptoms at all. When symptoms develop, they are most commonly the following:[2, 3, 24, 25, 26]

  • Abdominal pain
  • Weight loss
  • Malaise

Uncommonly, presenting signs and symptoms may include the following:

  • Migratory thrombophlebitis (Trousseau syndrome) or venous thrombosis as a result of direct invasion of the hepatic veins and inferior vena cava (IVC) or mass effect on the IVC[27]
  • Pain and fever simulating a hepatic abscess[28]
  • Obstructive jaundice due to biliary compression[29, 30]
  • Gynecomastia, due to aromatase production by fibrolamellar carcinoma cells and resultant conversion of circulating androgens to estrogens[24, 31, 32]

Other unusual presentations of fibrolamellar carcinoma have been summarized by Torbenson.[7]

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Physical

See the list below:

  • The most common physical finding is an abdominal mass or fullness due to hepatomegaly.[24]
  • Unlike patients with typical hepatocellular carcinoma, the stigmata of chronic liver disease and portal hypertension are usually absent in patients with fibrolamellar carcinoma.
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Causes

See the list below:

  • Specific risk factors for fibrolamellar carcinoma remain unidentified.
  • Unlike typical hepatocellular carcinoma (HCC), fibrolamellar carcinoma is not associated with hepatotoxins, cirrhosis, α1-antitrypsin deficiency, or hemochromatosis.
  • Less than 10-20% of cases of fibrolamellar carcinoma are associated with hepatitis B viral infection, but this may just be related to the high worldwide prevalence of hepatitis B infection.[7]
  • Fibrolamellar carcinoma is rarely associated with hepatitis C viral infection.
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Contributor Information and Disclosures
Author

Michael A Choti, MD, MBA, FACS Hall and Mary Lucile Shannon Professor and Chair, Department of Surgery, University of Texas Southwestern Medical Center

Michael A Choti, MD, MBA, FACS is a member of the following medical societies: American Association for the Study of Liver Diseases, American Surgical Association, International Hepato-Pancreato-Biliary Association, Americas Hepato-Pancreato-Biliary Association, American Society of Clinical Oncology, American College of Surgeons, Association for Academic Surgery, Society for Surgery of the Alimentary Tract, Society of Surgical Oncology, Society of University Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Hari Nathan, MD House Staff, Department of Surgery, The Johns Hopkins Hospital; Postdoctoral Fellow, Department of Surgery, The Johns Hopkins University School of Medicine

Hari Nathan, MD is a member of the following medical societies: American College of Surgeons, Association for Academic Surgery, Society for Surgery of the Alimentary Tract, Americas Hepato-Pancreato-Biliary Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

N Joseph Espat, MD, MS, FACS Harold J Wanebo Professor of Surgery, Assistant Dean of Clinical Affairs, Boston University School of Medicine; Chairman, Department of Surgery, Director, Adele R Decof Cancer Center, Roger Williams Medical Center

N Joseph Espat, MD, MS, FACS is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Surgeons, American Medical Association, American Society for Parenteral and Enteral Nutrition, American Society of Clinical Oncology, Americas Hepato-Pancreato-Biliary Association, Association for Academic Surgery, Central Surgical Association, Chicago Medical Society, International Hepato-Pancreato-Biliary Association, Pancreas Club, Sigma Xi, Society for Leukocyte Biology, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Surgical Oncology, Society of University Surgeons, Southeastern Surgical Congress, Southern Medical Association, Surgical Infection Society

Disclosure: Nothing to disclose.

Additional Contributors

Lodovico Balducci, MD Professor, Oncology Fellowship Director, Department of Internal Medicine, Division of Adult Oncology, H Lee Moffitt Cancer Center and Research Institute, University of South Florida Morsani College of Medicine

Lodovico Balducci, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Research, American College of Physicians, American Geriatrics Society, American Society of Hematology, New York Academy of Sciences, American Society of Clinical Oncology, Southern Society for Clinical Investigation, International Society for Experimental Hematology, American Federation for Clinical Research, American Society of Breast Disease

Disclosure: Nothing to disclose.

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Fibrolamellar carcinoma: Note the large tumor size in the background of a noncirrhotic liver.
CT scan showing fibrolamellar carcinoma with a large stellate central scar.
T1-weighted MRI of a fibrolamellar carcinoma in the left lobe of the liver.
T2-weighted MRI of a fibrolamellar carcinoma in the left lobe of the liver, demonstrating a heterogeneous appearance with a central scar.
Hematoxylin and eosin photomicrograph of fibrolamellar carcinoma. Note the thick fibrous lamellae within the tumor stroma.
Masson trichrome stain photomicrograph of fibrolamellar carcinoma depicting the collagen-containing lamellae within the tumor stroma.
 
 
 
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