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Case Reports
. 2019 Aug 21;1(2):175-178.
doi: 10.1016/j.jaccas.2019.05.031. eCollection 2019 Aug.

Fontan Liver Lesions: Not Always HCC

Christiane Haeffele  1   2 , Avin Aggarwal  3 , Glen Lutchman  3 , Gruschen R Veldtman  4 , Fred M Wu  5   6 , George K Lui  1   2
Affiliations
Case Reports

Fontan Liver Lesions: Not Always HCC

Christiane Haeffele et al. JACC Case Rep. .

Abstract

A 24-year-old Fontan procedure patient underwent surveillance liver cardiac magnetic resonance imaging. Findings were suggestive of hepatocellular carcinoma (HCC). Currently, HCC is diagnosed based on imaging alone. Given her otherwise reassuring clinical profile, she underwent liver biopsy. Pathology demonstrated focal nodular hyperplasia. This raises concern for overdiagnosis of HCC in Fontan patients without tissue confirmation. (Level of Difficulty: Advanced.).

Keywords: AASLD, American Association for Study of Liver Disease; AFP, alpha-fetoprotein; ALT, lanine aminotransferase; AST, aspartate aminotransferase; AV, atrioventricular; CT, computed tomography; FALD, Fontan associated liver disease; FNH, focal nodular hyperplasia; GS, glutamine synthetase; HCC, hepatocellular carcinoma; INR, international normalized ratio; MRI, magnetic resonance imaging; US, ultrasound; awareness; congenital heart defect; cyanotic heart disease; imaging; treatment.

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Figures

None
Graphical abstract
Figure 1
Figure 1
Hyperintense Reticulations Consistent With Fibrosis (A) Axial T2, fine T2 hyperintense reticulations consistent with fibrosis. (B) MR elastogram, heterogeneous in distribution but highest area measured was 3.5 kPa. Elastography of 3.5 kPa has been demonstrated to correlate with stage F0 to F1 fibrosis.
Figure 2
Figure 2
Arterial Phase Enhancement and Venous Phase Washout (A) Arterial phase shows hyperenhancement. Oval circle in A indicates a nodule that shows hyperenhancement during the arterial phase of the scan. (B) Venous phase shows slightly hypointense/washout with a suggestion of pseudocapsule.
Figure 3
Figure 3
Ductular Reaction With Varied Intensity at the Junction of Fibrous Septa With a Hepatocellular Component (A) Presence of ductular reaction with varied intensity at the junction of the fibrous septa with a hepatocellular component. Thick abnormal arteries are also present. (B) Evidence of copper-binding protein (demonstrated by Victoria blue stain) suggests FNH. (C) “Map-like” staining of hepatocytes. Glutamine synthetase (GS) staining of hepatocytic cytoplasm creates a “map-like” pattern, often surrounding hepatic veins, whereas GS staining is not expressed in hepatocytes close to fibrotic bands containing arteries and ductules. FNH = focal nodular hyperplasia.
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References

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