The Fontan operation is performed in patients with a single ventricle. As the systemic venous return is directly connected to the pulmonary circulation during this procedure, chronic hepatic congestion is induced, leading to Fontan-associated liver disease (FALD) including liver cirrhosis and hepatocellular carcinoma (HCC). In this report, we present a case of HCC diagnosed in a patient who underwent the Fontan operation 30 years ago. The patient underwent regular surveillance for FALD, which revealed a 4 cm-sized hepatic mass with elevated serum alpha-fetoprotein. After surgical treatment, there was no evidence of HCC recurrence during 3 years of follow-up. As the risk of HCC and Fontan-associated liver cirrhosis increases with the duration elapsed since the operation, regular surveillance should be emphasized. Serial follow-up of serum alpha-fetoprotein levels and abdominal imaging are necessary to achieve early and accurate diagnosis of HCC in post-Fontan patients.
Hepatocellular carcinoma (HCC) is heterogeneous in pathogenesis, phenotype and biological behavior. Various histopathological features of HCC had been sporadically described, and with the identification of common molecular alterations of HCC and its genomic landscape over the last decade, morpho-molecular correlation of HCC has become possible. As a result, up to 35% of HCCs can now be classified into histopathological variants, many of which have unique molecular characteristics. This review will provide an introduction to the variously described histopathological variants of HCC in the updated WHO Classification of Digestive System Tumors.
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Hepatocellular carcinoma (HCC) shows a poor prognosis with high recurrence rate even after
surgical resection. To improve prognosis of HCC patient, regular surveillance for high-risk
group is recommended, but cost-benefit of the surveillance under 40 years old Asian male
with hepatitis B infection is unclear. We share a 39-year-old male case which showed early
recurrence and rapid extrahepatic metastasis after surgical resection for single huge HCC.
Based on the pathologic finding, this case was diagnosed with ‘stemness’-related markerexpressing
HCC. Further molecular classification for HCC could be beneficial to estimate
individual risk for HCC recurrence and to predict prognosis.
Background/Aims Loss of liver fatty acid binding protein (LFABP) expression by immunohistochemistry
is a useful marker for the identification of hepatocyte nuclear factor 1α (HNF1α)-
inactivated hepatocellular adenomas; however, the expression status of LFABP in hepatocellular
carcinomas (HCCs) is still unclear. We aimed to investigate the expression status of LFABP
in HCCs and examine the clinicopathological characteristics of LFABP-negative HCCs. Methods Immunohistochemical stains LFABP, K19 (mouse monoclonal, Dako, Glostrup, Denmark)
and EpCAM (mouse monoclonal, Calbiochem, Darmstadt, Germany) were performed
on tissue microarray sections from 188 surgically resected HCCs, and the association between
LFABP expression status and the clinicopathological features, survival and “stemness”-related
marker expression status were analyzed. Results Loss of LFABP expression was noted in 30 (16%) out of 188 HCCs. LFABP-negative
HCCs were associated with a decreased recurrence-free survival (LFABP-negative: 17.0 ± 4.84
months [95% confidence interval [CI]: 7.5–26.5 months] versus LFABP-positive: 51.0 ± 8.7
months [95% CI: 34.0–68.0 months]; P=0.004). HCCs with LFABP expression loss were more
frequently larger and showed more frequent vascular invasion, although not statistically significant;
and an inverse correlation was seen between LFABP expression and K19 expression
status (P=0.001). Conclusions Loss of LFABP expression is seen in HCCs, and is associated with a decreased
recurrence-free survival.
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