Fibrolamellar hepatocellular carcinoma

Fibrolamellar hepatocellular carcinoma is a distinct variant of generic hepatocellular carcinoma (HCC), with different demographics and risk factors (see epidemiology).

Typically these tumours occur in young adults (20 to 40 years of age) without gender predilection. Unlike HCCs they do not have an association with cirrhosis, alcoholism or hepatitis B/C infection 2.

Presentation is nonspecific, with constitutional symptoms and occasionally gynaecomastia due to elevated oestrone levels 1. Hepatomegaly is usually evident as these masses are typically large.

These tumours are well differentiated and well circumscribed, with dense fibrotic background 2. The tumour cells are arranged in cords that are separated by sheetlike fibrous bands arranged in a parallel or lamellar distribution 4: hence the name, fibro-lamellar. 

Fibrolamellar carcinomas often do not produce alpha fetoprotein (AFP) 5.

Ultrasound
  • variable sonographic appearance
  • contrast-enhanced ultrasound 6
    • arterial phase:
      • heterogeneous enhancement
    • portal venous phase:
      • decreased echogenicity relative to background liver ("wash out")
CT

Fibrolamellar HCCs typically are single large tumours with dense fibrotic bands forming a central scar (seen in ~75% of cases) 1, which make it resemble focal nodular hyperplasia (FNH). A few small calcifications are seen in 35-65% of cases 1. Regional (hepatic hilum) nodal enlargement is seen in ~50% of cases. Enhancement is usually arterial. The central scar typically shows persistent enhancement on delayed contrast enhanced CT. 

MRI

The central scar, when present, is usually hypointense on all sequences. Occasionally it may be T2 hyperintense mimicking FNH 1. Fibrolamellar HCCs do not contain fat, and thus do not lose signal on out of phase imaging 7

Signal characteristics
  • T1: typically iso to hypointense to the liver
  • T2: hypo to slightly hyperintense
  • T1 C+ (Gd)
    • arterial phase: heterogeneous enhancement
    • portal venous / delayed phase: iso to hypointense
Nuclear medicine

Technetium-99m sulphur colloid scans (taken up by Kupffer cells) are useful as these tumours will not accumulate the agent, whereas FNH does.

Fibrolamellar carcinoma is less aggressive than HCC but usually, due to lack of symptoms until it becomes sizeable, stage at presentation tends to be advanced. Also, It often does not produce AFP 5.

Resection is the treatment of choice, with a resulting 5-year survival of 76%. This compares favourably to generic HCC which, even when resectable, have a 5-year survival of only 37-56% 1.

If unresectable, this tumour is fatal.

General imaging differential considerations include:

Hepatobiliary pathology
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Article Information

rID: 6994
Synonyms or Alternate Spellings:
  • Fibrolamellar carcinoma
  • Fibrolamellar HCC
  • Fibrolamellar hepatocellular carcinomas
  • Fibrolamellar carcinomas

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