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.
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.
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.
- typically iso intense to the liver
- hypo to slightly hyperintense
- may demonstate hypo intense central scar 1
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.
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
- 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
- 1. Smith MT, Blatt ER, Jedlicka P et-al. Best cases from the AFIP: fibrolamellar hepatocellular carcinoma. Radiographics. 28 (2): 609-13. doi:10.1148/rg.282075153 - Pubmed citation
- 2. Kumar V, Abbas AK, Fausto N et-al. Robbins and Cotran pathologic basis of disease. W B Saunders Co. (2005) ISBN:0721601871. Read it at Google Books - Find it at Amazon
- 3. Lubbers PR, Ros PR, Goodman ZD et-al. Accumulation of technetium-99m sulfur colloid by hepatocellular adenoma: scintigraphic-pathologic correlation. AJR Am J Roentgenol. 1987;148 (6): 1105-8. AJR Am J Roentgenol (abstract) - Pubmed citation
- 4. Mclarney JK, Rucker PT, Bender GN et-al. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics. 19 (2): 453-71. Radiographics (full text) - Pubmed citation
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