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fibrolamellar hepatocellular carcinoma
Thursday 19 January 2006
fibrolamellar variant of hepatocellular carcinoma, FHCC, Fibrolamellar HCC
Fibrolamellar carcinoma has a distinctive morphology and immunophenotype, including cytokeratin 7 and CD68 co-expression.
Recently, fibrolamellar carcinomas were found to harbor a characteristic somatic gene fusion, DNAJB1-PRKACA.
FISH for the PRKACA rearrangement is a clinically useful tool to confirm the diagnosis of fibrolamellar carcinoma, with high sensitivity and specificity.
Images
Fibrolamellar (hepatocellular) carcinoma, DNAJB1-PRKACA fusion positive
Touchprep of Fibrolamellar HCC
Synopsis
Predominantly young patients without cirrhosis
Account for almost 50% of liver cell carcinomas in the US in patients @<@35 years
Macroscopy
Fibrolamellar carcinoma is large, well circumscribed, multinodular, and yellow.
Microscopy
Fibrolamellar carcinoma shows tumor lobules separated by wide fibrous bands.
oncocytic appearance of the tumor cells.
polygonal tumoral cells with fibrous stroma
fibrosis arranged in a lamellar fashion around the neoplastic hepatocytes.
Tumor cells are polygonal and deeply eosinophilic
tumor lobules separated by wide fibrous bands
oncocytic appearance of the tumor cells.
Immunochemistry
express cytokeratins 8 (CK8) and 18 (CK18) (like those of conventional liver cell carcinoma), but also CK7 and (to a lesser extent) CK19
Ultrastructure
numerous mitochondria, in keeping with their oncocytic appearance at the light microscopic level
bile canaliculi-like lumina
sometimes neurosecretory-type dense-core granules
Collagen deposited around the tumor cells is predominantly composed of types I, III, and V
Pathogenesis
It may be pathogenetically related to focal nodular hyperplasia (FNH) because of architectural similarities and occasional coexistence of the two entities.
Associations
hepatocellular adenoma (14736278)
Fanconi anemia
Molecular biology
DNAJB1-PRKACA fusion gene in 100% of tumors (24578576)
- Detection of a Recurrent DNAJB1-PRKACA Chimeric Transcript in Fibrolamellar Hepatocellular Carcinoma
overexpression of genes in the RAS, MAPK, PIK3, and xenobiotic degradation pathways (17367606)
EGFR overexpression (16564914)
gains
losses
Cytogenetics
1q anomaly (8986150)
near triploid karyotype (8986150)
complex karyotype (8640730, 11338475, 12768875)
See also
hepatic tumors
Open references
Molecular testing for the clinical diagnosis of fibrolamellar carcinoma. Graham RP, Yeh MM, Lam-Himlin D el al. Mod Pathol. 2018 Jan;31(1):141-149. doi : 10.1038/modpathol.2017.103 PMID: 28862261
Hepatocellular Carcinoma, Fibrolamellar Variant: Diagnostic Pathologic Criteria and Molecular Pathology Update. A Primer. 2016. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4808818/
Detection of a Recurrent DNAJB1-PRKACA Chimeric Transcript in Fibrolamellar Hepatocellular Carcinoma. 2015. (24578576) http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4286414/
References
Kannangai R, Vivekanandan P, Martinez-Murillo F, Choti M, Torbenson M. Fibrolamellar carcinomas show overexpression of genes in the RAS, MAPK, PIK3, and xenobiotic degradation pathways. Hum Pathol. 2007 Apr;38(4):639-44. PMID: 17367606
Buckley AF, Burgart LJ, Kakar S. Epidermal growth factor receptor expression and gene copy number in fibrolamellar hepatocellular carcinoma. Hum Pathol. 2006 Apr;37(4):410-4. PMID: 16564914
