What is fibrolamellar hepatocellular carcinoma?

Updated: Sep 05, 2019
  • Author: W Ross Stevens, MD; Chief Editor: John Karani, MBBS, FRCR  more...
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Answer

Answer

Fibrolamellar hepatocellular carcinoma, or fibrolamellar carcinoma, is a rare primary malignant neoplasm of the liver that constitutes 0.8-16% of all hepatocellular malignancies. [1, 2]  It typically affects persons between 5 and 35 years, but there appears to be 2 peak incidences, at age 10-30 years and at 70-79 years. [3, 4, 5] Fibrolamellar carcinoma has distinctive clinical, histologic, [6, 7] and radiographic features that distinguish it from the relatively more common hepatocellular carcinoma (HCC). It often presents with vague abdominal pain, nausea, abdominal fullness, malaise, and weight loss. [8] Fibrolamellar carcinoma occurs in a younger population than does HCC, and it is typically not associated with underlying liver disease or elevated serum levels of alpha-fetoprotein tumor markers. [9] Also, fibrolamellar carcinoma may have a slightly better prognosis than HCC. [10, 11]   

(See the images of fibrolamellar hepatocellular carcinoma below.)

Contrast-enhanced computed tomography (CT) scan of Contrast-enhanced computed tomography (CT) scan of fibrolamellar carcinoma demonstrates a large, heterogeneously enhancing mass in the right lobe of the liver, with a hypoattenuating central scar and punctate, central calcification.
Digital subtraction arteriogram (obtained by injec Digital subtraction arteriogram (obtained by injection of the celiac axis) demonstrates fibrolamellar carcinoma; the carcinoma appears as an area of hypervascularity and tumor blush in the inferior aspect of the liver.

Perhaps because of the younger ages of the patients and the lack of coexisting cirrhosis, patients with fibrolamellar carcinoma are often treated aggressively. Resection of large tumor masses, of metastatic disease, and even of recurrent disease can extend patient survival.


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