Clinicopathological Features of Two Extremely Rare Hepatocellular Carcinoma Variants: a Brief Review of Fibrolamellar an
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REVIEW ARTICLE
Clinicopathological Features of Two Extremely Rare Hepatocellular Carcinoma Variants: a Brief Review of Fibrolamellar and Scirrhous Hepatocellular Carcinoma Emine Turkmen Samdanci 1 & Ayse Nur Akatli 1 & Nese Karadag Soylu 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose We aimed to distinguish between fibrolamellar hepatocellular carcinoma and scirrhous hepatocellular carcinoma histopathologically. Methods and results In this review, fibrolamellar hepatocellular carcinoma and scirrhous hepatocellular carcinoma two specific and rare variants of hepatocellular carcinoma, which are difficult to diagnose histopathologically are discussed. Conclusion The clinical, radiological, gross, histopathological, immunohistochemical, and molecular features of these two tumors, which are defined by the World Health Organization (WHO), are mentioned. Keywords Fibrolamellar hepatocellular carcinoma . Scirrhous hepatocellular carcinoma . Tumors
Introduction
Clinical Characteristics
Hepatocellular carcinoma (HCC) is a malignant epithelial tumor of the liver, originated from hepatocytes. Although it has a primary hepatic differentiation, it is a heterogeneous tumor with several subtypes [1, 2]. It is important to identify the subtypes of HCC for many reasons. Thus, the tumors in the liver have given a clear name, and the subtyping which explains prognostic significance can be made. And, the identification of some subtypes gives information about the genetic changes that may contribute to the development of these tumors [3]. Fibrolamellar hepatocellular carcinoma (FLHCC) and scirrhous hepatocellular carcinoma (SHCC) are morphologically similar due to their abundant fibrosis and cellular features. In this article, a brief review of FLHCC and SHCC with fibrotic stroma, which is difficult to differentiate histomorphologically and which actually includes differences in clinical prognosis and treatment, has been made [2, 4].
Fibrolamellar HCC, which is often seen in western countries, is rare and constitutes almost 0.5–9% of primary liver cancers [5–7]. Fibrolamellar HCC is seen generally in late adolescents and adults (2nd and 3rd decades). Cirrhosis and typical risk factors for HCC such as viral hepatitis, alcohol abuse, metabolic and storage diseases are absent [8–11]. Generally, serum alpha-fetoprotein (AFP) levels are normal and slightly increased in a few cases [12, 13]. It has a better prognosis compared with conventional HCC. This is because there is no cirrhosis and also the tumor is resectable [14–16]. The patients commonly have abdominal pain, cachexia, hepatomegaly, and rarely, fever, jaundice (biliary compression), gynecomastia, and venous thrombosis that may suggest hepatic abscess as a diagnosis [17]. Scirrhous HCC constitutes nearly 0.2–4.2% of HCCs [5, 18]. Scirrhous HCC is seen in the older age group and is more common in cirrhotic cases than in non-cirrhotic cases. Many studies have shown that the level of AFP in SHCC does not differ significantly from conventio
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