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Fibrolamellar hepatocellular carcinoma

Dr Maxime St-Amant and Dr Frank Gaillard et al.

Fibrolamellar hepatocellular carcinoma is a distinct variant of generic hepatocellular carcinoma. It has different demographics and risk factors.

Demographics and clinical presentation

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 hepatitic B / C infection ².

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

Pathology

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 distribution⁴: hence the name, fibro-lamellar.

Radiographic features

CT

Fibrolamellar carcinomas 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.

MRI

T1
- typically iso intense to the liver
T2
- hypo to slightly hyperintense
- may demonstate hypo intense central scar ¹

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.

Treatment and prognosis

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

If unresectable, then this tumour is fatal.

Differential diagnosis

hepatocellular carcinoma (HCC)
- different demographics
- less likely to have calcification or central scar
focal nodular hyperplasia (FNH)
- uptake of Tc99m sulphur colloid usually present
- scar is often hyper intense on T2
hepatic adenoma
- uptake of Tc⁹⁹m sulphur colloid present in up to 25% of cases ³
hepatic metastasis
large liver haemangioma
- peripheral pooling, delayed central filling in with contrast
- may also have central scar if large